Which medications in the drug class Chelators are used in the treatment of Wilson Disease?

Updated: Feb 14, 2019
  • Author: Richard K Gilroy, MBBS, FRACP; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Chelators

Chelating agents bind excess copper. Ammonium tetrathiomolybdate is an investigational chelating drug used at the University of Michigan as an initial treatment for patients who present with neurologic or psychiatric manifestations. This drug works as a chelating agent and as an inhibitor of copper absorption from the GI tract. [24]

Penicillamine (Cuprimine, Depen)

Penicillamine forms soluble complexes with metals excreted in urine. It was the drug of choice before newer regimens were available. Because of extensive toxicities, alternative agents are used. It must be administered with pyridoxine 25 mg by mouth daily.

Trientine (Syprine)

Trientine is an effective oral chelator used to induce cupruresis. It is useful for patients who cannot tolerate penicillamine. It is indicated in Wilson disease if the initial presentation is hepatic. It should be administered with zinc.

Dimercaprol (BAL in Oil)

Dimercaprol is for refractory cases of Wilson disease that are not responding to first- or second-line chelation treatment.


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