Menkes Disease Medication

Updated: Oct 29, 2014
  • Author: Celia H Chang, MD; Chief Editor: Amy Kao, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications. Infants that carry the mutation must be identified and treated very early in life before irreparable neurodegeneration occurs.

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Trace Elements

Class Summary

Copper histidine used in this class has been shown to prevent neurodegeneration when administered early in life.

Copper histidine

Copper histidine is a copper replacement administered parenterally to avoid absorption through the gastrointestinal tract. Patients with a genetic abnormality that are still able to produce limited amounts of enzymes, involved in copper metabolism, may receive benefit from early treatment. A dose of 350-500 µg IV/SC qDay or qod has been shown to be effective.

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