What are the stages 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

The natural history of Wilson disease may be considered in four stages, as follows:

  • Stage I - The initial period of accumulation of copper within hepatic binding sites

  • Stage II - The acute redistribution of copper within the liver and its release into the circulation

  • Stage III - The chronic accumulation of copper in the brain and other extrahepatic tissue, with progressive and eventually fatal disease

  • Stage IV - Restoration of copper balance by the use of long-term chelation therapy


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