Wilson disease is a genetic disorder that affects copper storage, leading to liver failure and neurologic deterioration.

Researchers at the University Hospital of Heidelberg, in Germany, studied the long-term outcomes copper chelators vs zinc salts.

First, the details.

• The medical records of 288 patients analyzed over about 17 years.
• Adherence to therapy, survival, treatment failure, and adverse events from different treatment were compared.
  • Chelators
  • Zinc
  • Combination treatment
• Liver treatment failure was defined as an increase in activity of liver enzymes, with an increase in urinary copper excretion.

And, the results.

• Liver and neuropsychiatric symptoms occurred in 68% and 34% of patients, respectively.
• Liver treatment failure occurred significantly more often from zinc therapy than from chelator therapy.
• Actuarial survival, without transplantation, showed a significant advantage for chelating agents vs zinc.
• Patients who did not respond to zinc therapy showed hepatic improvement after reintroduction of a chelating agent.

The bottom line?

The authors concluded, “Treatments with chelating agents or zinc salt are effective in most patients with Wilson disease; chelating agents are better at preventing hepatic deterioration.”

“It is important,” they continue, “to identify patients who do not respond to zinc therapy and have increased activities of liver enzymes, indicating that a chelating agent should be added to the therapeutic regimen.”

Mayoclinic.com has a succinct review that present background on Wilson’s disease, and places these treatment options into perspective.

3/30/11 20:50 JR