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abnormal
copies
of
the
ATP7B
genes,
one
from
each
parent.
Those
with
only
one
abnormal
copy
also
carry
this
disease,
however
without
symptoms.
Actually,
a
small
amount
of
copper
is
essential
for
our
body,
however
too
much
of
it
will
be
very
harmful
and
poisonous.
This
excess
copper
then
build
up
in
the
organs:
brain,
liver,
eyes,
and
kidneys,
and
it
will
damage
them.
Also,
neurological
problems
have
a
potential
to
occur
and
over
time,
it
may
cause
death.
Luckily,
there
are
therapies
to
treat
this
disease.
Firstly,
they
should
reduce
the
amount
of
copper
intake,
which
means
that
they
mustn’t
consume
foods
or
water
with
high
copper
concentration
like
liver,
shellfish,
nuts,
and
even
multivitamins
that
contain
copper.
Secondly,
they
should
remove
the
excess
copper
in
their
body
and
in
this
case,
science
is
notably
used.
Nowadays,
through
research
in
science
doctors
found
two
drugs:
D-‐penicillamine
(Cuprimine)
and
trientine
(Syprine)
to
help
the
removal
of
excess
One
of
the
patient
that
carry
this
disease.
copper.
These
two
drugs
help
to
release
the
excess
http://www.nature.com/nrne copper
into
the
bloodstream,
which
is
then
filtered
by
urol/journal/v2/n9/fig_tab/n
cpneuro0291_F3.html
1
Copper
is
a
metallic
element,
which
is
a
necessary
nutrient
for
normal
growth
and
development.
An
average
diet
provides
about
2mg
of
copper
per
day.
1
the
kidneys
and
excreted
as
urine.
However,
it
is
without
disadvantage.
People
using
these
drugs
will
suffer
from
neurological
symptoms,
fever,
rash,
and
bone
marrows.
Another
therapy
is
by
using
zinc2
as
in
zinc
acetate
(Galzin).
It
blocks
the
digestive
system
tract’s
absorption
of
copper
from
the
food
and
it
doesn’t
have
any
side
effect
at
all.
Thirdly,
they
should
treat
any
symptoms
and
damage
that
occur
to
them.
Nevertheless,
this
treatment
is
only
pain
relieving
and
intended
to
maintain
copper
balance
–
not
to
eliminate
the
disease.
Thus,
a
life-‐long
commitment
is
needed.
This
therapy
without
doubt
will
have
a
massive
impact
to
the
society
–
the
social
aspect.
It
will
definitely
lift
up
the
motivation
of
those
people
who
suffer
from
this
disease
to
live
and
thus,
creating
a
better
and
joyful
society.
In
the
same
time,
it
also
plays
a
significant
role
to
the
economical
aspect.
This
therapy,
as
you
see,
is
quite
expensive
(it
costs
$77.50
for
100
tablets
of
D-‐penicillamine3
and
$1025
for
100
tablets
of
trientine4).
So,
not
all
people
can
afford
to
buy
these
drugs,
and
hence
not
all
of
them
can
treat
his/her
disease.
Christopher W / 9T
2
Zinc
is
an
effective
anti-‐copper
treatment.
Zinc
acts
by
stimulating
the
production
of
metallothionein
in
intestinal
cells.
This
metallothionein
binds
to
the
copper
from
foods
and
from
gastrointestinal
track
secretions
and
therefore
prevents
its
absorption
into
the
body.
3
According
to
http://www.universaldrugstore.com/medications/Cuprimine/250mg
4
According
to
http://www.internationalpharmacy.com/en/products/details/093344?IPS=dd8076ac342a22f005ef83db45817cd
2
Reference:
• http://www.genome.gov/27532725
• http://digestive.niddk.nih.gov/ddiseases/pubs/wilson/
• www.digestive.niddk.nih.gov.
• www.eurowilson.org
• Frydman M. Genetic aspects of Wilson ́s disease. J Gas- troenterol
Hepatol 1990; 5: 483-90
• Yarze JC et al. Wilsonʼs disease: current status. Am J Med 1992; 92: 643-
54
• Walshe JM. Treatment of Wilsonʼs disease with trientine (triethylene
tetramine) dichlo- ride. Lancet 1982; 1: 643-647
3