Which medications in the drug class Chelating agents are used in the treatment of Fanconi Syndrome?

Updated: Feb 09, 2018
  • Author: Sahar Fathallah-Shaykh, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

Chelating agents

These agents inhibit a toxin by reacting with it to form less active or inactive complex.

D-penicillamine (Cuprimine, Depen)

Recommended for removal of excess copper in patients with Wilson disease. In vitro, 1 atom of copper combines with 2 molecules of penicillamine; 1 g of penicillamine is expected to cause excretion of approximately 200 mg of copper. In practice, however, only about 1% of this amount excreted. Determine dosage by measurements of urinary copper excretion and free copper in the serum.

Trientine hydrochloride (Syprine)

Use in patients who are intolerant to penicillamine. Clinical experience limited. Unlike penicillamine, does not contain a sulfhydryl group, making it unable to chelate cystine; therefore, use only to treat Wilson disease. Administer on empty stomach and swallow capsules whole with water.


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