Wilson's
Disease
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Wislons disease is caused by the accumulation
of copper in the liver. see
Copper Toxicity
Copper toxicity is a common symptom of Candidia
see our Candidia pages
Authors Full Name Hoogenraad, Tjaard Ubbo.
Institution University Department of Neurology,
UMC-Utrecht, Heidelberglaan 100, 3584
CX, The Netherlands. tu.hoogenraad@planet.nl
Title Paradigm shift in treatment of Wilson's
disease: zinc therapy now treatment of
choice.
Source Brain & Development. 28(3):141-6,
2006 Apr.
" Abstract Zinc therapy has replaced
penicillamine as first-line therapy for
Wilson's disease. New guidelines reflect
the paradigm shift in treatment that has
occurred in recent years. In the old paradigm,
Wilson's disease was seen as genetic disorder
associated with the accumulation of copper
in the liver and in other organs once
the liver had become overloaded with copper.
When left untreated, the disease was regarded
as uniformly fatal. The old treatment
guidelines advised, 'decoppering' with
penicillamine because this chelating agent
was considered effective in restoring
most patients to health. Before the start
of treatment, patients were warned that
their symptoms could worsen during the
first weeks or months of therapy, so as
to prevent them from abandoning penicillamine
therapy in dismay. In the new paradigm,
Wilson's disease is seen as a hereditary
disorder associated with copper intoxication.
The essence of symptomatic Wilson's disease
is poisoning by free copper in the blood,
that is, by copper that is not bound to
ceruloplasmin. This form of copper is
toxic, whereas accumulated copper and
copper that is bound to ceruloplasmin
or metallothionein is not. The treatment
of symptomatic Wilson's disease is no
longer aimed at 'decoppering', the removal
of accumulated copper, but at the normalization
of the free copper concentration in blood,
to reverse the copper poisoning. This
can be achieved safely and effectively
with zinc therapy. Zinc induces metallothionein,
a highly effective detoxification protein
that binds copper. Oral zinc therapy leads
to storage of metallothionein-bound copper
in the mucosa of the gut and to the excretion
of copper via the stools. New treatment
guidelines advise against the use of chelating
agents as initial treatment because they
may aggravate copper intoxication and
cause iatrogenic deterioration." |
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