Wislons disease is caused by the
accumulation of copper in the liver.
Copper toxicity is a common symptom
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Authors Full Name Hoogenraad,
Institution University Department
of Neurology, UMC-Utrecht, Heidelberglaan
100, 3584 CX, The Netherlands.
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."