Wilson Disease and Trientine Dihydrochloride

Wilson Disease, also known as hepatolenticular degeneration, is a rare inherited autosomal recessive disorder of copper metabolism, resulting in copper toxicity and dysfunction of several organs such as the liver, central nervous system, eyes and kidneys. The disease has a gene frequency of 1 in 90-150 and an incidence of 1 in 30,000.

Untreated Wilson Disease is universally fatal, with most patients dying from liver disease and a minority from complications of progressive neurologic disease. The goal of drug therapy is to remove excess copper from the body and prevent ongoing copper accumulation and deposition. Treatment for WD is lifelong. Liver transplantation which corrects the underlying hepatic defect in WD is reserved for severe or resistant cases only

Wilson Disease is a very treatable condition. Trientine dihydrochloride, a chelating agent, is indicated for the treatment of Wilson Disease in patients who are intolerant of D-Penicillamine therapy or have clinical features indication potential intolerance.

Univar has been manufacturing and supplying Trientine dihydrochloride for the treatment of Wilson Disease patients since 1985.


This website is intended for information purposes only.

Each patient is strongly advised to consult with their physician about their individual cases.

Latest News

EASL publishes Clinical Practice Guidelines for Wilson Disease
4th March 2014
The European Association for the study of the Liver (EASL) has published a Clinical Practice Guidelineā€¦ more...
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