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Alpha-1
antitrypsin
protects
the
delicate
tissues
of
the
lungs
by
binding
to
neutrophil
elastase,
a
protein
released
by
white
blood
cells
which
digests
bacteria
and
other
foreign
objects
in
the
lungs.
When
a
person
who
is
deficient
of
Alpha-1
antitrypsin
inhales
irritants,
or
contracts
a
lung
infection,
the
neutrophil
elastase
released
in
the
lungs
continues
digesting
irritants
unchecked,
eventually
destroying
healthy
lung
tissue.
The
eventual
result
of
the
destruction
of
healthy
lung
tissue
by
neutrophil
elastase is
emphysema.
However,
Alpha-1
Antitrypsin
Deficiency
emphysema
(also
known
as
"genetic"
or
"inherited"
emphysema)
is
different
than
emphysema
caused
by
smoking
("acquired"
emphysema).
In
emphysema
caused
by
smoking
the
damage
usually
affects
the
upper
portion
of
the
lungs.
In
the
Alpha-1
patient,
the
lower
regions
of
the
lungs
are
first
affected.
With
either
cause,
the
lungs
are
hyperinflated
due
to
air
trapping
caused
by
the
destruction
of
the
lung
tissue,
and
the
diaphragms
are
flattened
due
to
the
hyperinflation
of
the
lungs.
Emphysema
is a
lung
disease
caused
by
the
destruction
of
the
delicate
walls
of
small
air
sacs
(alveoli).
With
this
destruction,
air
sacs
lose
their
elasticity
and
form
larger,
inefficient
sacs
that
cannot
properly
exchange
oxygen
and
carbon
dioxide
with
the
bloodstream.
In
addition,
it
becomes
harder
to
breathe
since
each
drawn
breath
inflates
the
lungs,
but
the
lungs
do
not
return
to
normal
with
the
exhaled
breath.
This
causes
air
to
become
trapped,
leading
to
over-inflation
of
the air
sacs.
Emphysema
caused
by
Alpha-1
is a
progressive
disease-the
destructive
action
continues
until
the
lungs
can
no
longer
bring
in
oxygen
to
the
bloodstream.
Alpha-1
emphysema
usually
causes
symptoms
in
people
while
they
are
in
their
30's
or
40's.
While
there
is
no
cure
for
Alpha-1,
there
are
treatments
available
for
the
symptoms,
as
well
as
augmentation
therapy,
which
may
slow
down
or
halt
the
destruction.
Patients
with
more
severe
or
advanced
Alpha-1
lung
disease
may
be
candidates
for
lung
transplantation.
Many
people
with
Alpha-1
also
have
chronic
bronchitis.
With
this,
the
lung
lining
becomes
swollen
and
congested
with
mucus,
restricting
air
flow.
The
bronchi
(air
passages)
often
go
into
bronchospasms,
which
are
contractions
of
the
muscles
which
further
reduce
air
flow.
This
often
results
in a
chronic
cough.
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