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In a recent study, zinc monotherapy, an inhibitor of intestinal copper uptake, was not as effective as chelating agents in the treatment of Wilson disease (NEJM JW Gastroenterol May 27 2011). However, information is lacking on which chelating agent is best. D-penicillamine has been in clinical use the longest, but trientine has been shown to have fewer adverse effects.
To compare the safety and efficacy of these chelator-based therapies for Wilson disease, researchers retrospectively analyzed data from 405 patients, most of whom were treated at tertiary centers in Germany and Austria. Neurologic and hepatic outcomes were assessed periodically up to 48 months, and median follow-up of discontinuation due to adverse events was 13.3 years.
A tota…