IN THE TREATMENT OF WILSON'S DISEASE...An alternative option
Wilson’s disease (WD), 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.The worldwide prevalence of WD is estimated to be one in 30,000 individuals.2
The condition can be 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 WD who are intolerant of the first-line treatment, penicillamine.3
INDICATIONS AND USAGE
Syprine® (trientine hydrochloride) is indicated in the treatment of patients with Wilson’s disease who are intolerant of penicillamine. Clinical experience with Syprine is limited and alternate dosing regimens have not been well-characterized; all endpoints in determining an individual patient’s dose have not been well defined. Syprine and penicillamine cannot be considered interchangeable. Syprine should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects.
 
Unlike penicillamine, Syprine is not recommended in cystinuria or rheumatoid arthritis. The absence of a sulfhydryl moiety renders it incapable of binding cystine and, therefore, it is of no use in cystinuria. In 15 patients with rheumatoid arthritis, Syprine was reported not to be effective in improving any clinical or biochemical parameter after 12 weeks of treatment.
 
Syprine is not indicated for treatment of biliary cirrhosis.
IMPORTANT SAFETY INFORMATION
  • Syprine is contraindicated in patients hypersensitive to Syprine or any components of the formulation. Patients should be observed closely for signs of possible hypersensitivity.
  • Patients receiving Syprine should remain under regular medical supervision throughout the period of drug administration. Patients (especially women) should be closely monitored for evidence of iron deficiency anemia.
  • The treatment can be monitored by the determination of free copper in the serum. Therapy may be monitored with a 24-hour urinary copper analysis periodically (i.e., every 6-12 months).
  • Patients should be directed to take Syprine on an empty stomach, at least one hour before meals or two hours after meals 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, the patient should have his temperature taken nightly, and he should be asked to report any symptom such as fever or skin eruption.
  • In general, mineral supplements should not be given since they may block the absorption of Syprine.
  • Syprine should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus.
  • The following adverse reactions have been reported in a clinical study: iron deficiency, systemic lupus erythematosus. In addition, dystonia, muscular spasm, myasthenia gravis have been reported in marketed use.
Please click here to see full Prescribing Information for Syprine Capsules.
References
  1. Gaffney D, Fell GS, O'Reilly DS. ACP Best Practice No 163. Wilson's disease: acute and presymptomatic laboratory diagnosis and monitoring. J Clin Pathol. 2000;53(11):807-812.
  2. European Association for Study of Liver. EASL Clinical Practice Guidelines: Wilson's Disease. J Hepatol. 2012;56(3):671-685.
  3. Syprine [package insert]. Bridgewater, NJ: Valeant Pharmaceuticals North America LLC; 2014.

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