Rare Diseases Symptoms Automatic Extraction
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A random Abstract
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Complement system is not activated in primary biliary cirrhosis.
[benign recurrent intrahepatic cholestasis]
There
is
controversial
evidence
suggesting
that
the
classical
pathway
of
complement
system
is
chronically
activated
in
primary
biliary
cirrhosis
(
PBC
)
and
that
complement
activation
may
be
important
in
development
of
bile
duct
injury
.
We
have
reevaluated
this
issue
by
measuring
by
-products
of
complement
activation
such
as
C
4
a
,
C
3
a
,
Bb
,
and
terminal
complement
complexes
(
SC
5
b
-
9
)
in
plasma
of
44
PBC
patients
with
sensitive
methods
not
previously
used
to
detect
complement
activation
in
this
disease
.
Age-matched
healthy
women
and
patients
with
chronic
hepatitis
of
different
etiology
were
studied
as
controls
.
We
found
that
PBC
patients
have
normal
C
4
a
concentrations
.
This
finding
argues
strongly
against
chronic
classical
pathway
activation
.
Although
a
minor
increase
of
C
3
a
levels
was
observed
in
a
minority
of
PBC
patients
,
the
C
3
a
/
C
3
ratio
,
an
index
used
to
evaluate
the
extent
of
native
protein
conversion
,
was
remarkably
similar
in
all
groups
.
Potentially
lytic
terminal
complement
complexes
were
not
increased
.
PBC
patients
had
normal
Bb
plasma
levels
,
indicating
that
the
alternative
pathway
is
also
not
activated
.
C
3
concentration
was
higher
in
PBC
patients
than
in
healthy
subjects
and
in
chronic
hepatitis
patients
,
particularly
in
the
early
stages
of
the
disease
.
C
3
and
C
4
concentrations
became
lower
in
PBC
and
chronic
hepatitis
with
the
progression
of
the
disease
.
The
increase
of
C
3
concentration
in
PBC
does
not
reflect
liver
inflammation
,
since
serum
levels
of
C-
reactive
protein
are
normal
.
We
found
high
serum
C
3
levels
in
patients
with
rare
chronic
cholestatic
syndromes
without
superimposed
infections
and
observed
that
serum
C
3
levels
paralleled
those
of
bilirubin
in
a
patient
with
benign
recurrent
intrahepatic
cholestasis
.
In
conclusion
,
our
data
indicate
that
complement
is
not
activated
in
PBC
and
that
the
increase
of
serum
C
3
levels
is
related
to
cholestasis
.
Diseases
Validation
Diseases presenting
"chronic hepatitis"
symptom
benign recurrent intrahepatic cholestasis
typhoid
waldenström macroglobulinemia
This symptom has already been validated
