Rare Diseases Symptoms Automatic Extraction
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Primary sclerosing cholangitis: outcome of patients undergoing restorative proctocolecetomy for ulcerative colitis.
[carcinoma of the gallbladder]
The
prevalence
of
primary
sclerosing
cholangitis
(
PSC
)
among
patients
with
ulcerative
colitis
needing
proctocolectomy
is
about
12
%
.
The
study
aim
was
to
evaluate
the
progression
of
the
liver
disease
after
surgery
.
PSC
progression
in
68
patients
with
UC
after
restorative
proctocolectomy
was
evaluated
after
a
median
follow-up
of
11
years
(
range
0
to
21
)
.
Magnetic
resonance
imaging
(
MRI
)
of
the
liver
,
histological
examination
of
a
core
needle
liver
specimen
,
and
liver
function
tests
were
used
in
addition
to
clinical
history
.
Of
the
68
patients
,
30
participated
in
follow-up
examinations
.
Ductal
changes
in
MRI
suggesting
a
diagnosis
of
PSC
occurred
in
21
(
72
%
)
of
them
.
One
carcinoma
of
the
gallbladder
was
found
in
MRI
.
Histopathologic
changes
suggesting
PSC
were
observable
in
15
(
50
%
)
patients
.
Compared
to
stage
in
peroperative
biopsies
taken
at
proctocolectomy
,
PSC
stage
increased
in
four
(
13
%
)
patients
,
decreased
in
15
(
50
%
)
,
and
remained
unchanged
in
11
(
37
%
)
.
Immunohistochemical
staining
for
cytoceratin-
7
in
hepatocytes
was
positive
in
nine
(
30
%
)
indicating
cholestasis
.
After
IPAA
surgery
,
five
patients
underwent
liver
transplantation
at
1
,
1
,
5
,
6
,
and
11
years
,
respectively
.
Of
the
68
,
six
patients
have
,
to
date
,
developed
cholangiocarcinoma
.
Progression
of
PSC
in
patients
with
minor
ductal
changes
at
the
time
of
restorative
proctocolectomy
is
unlikely
.
Those
patients
with
more
advanced
disease
at
surgery
are
at
risk
for
disease
progression
and
liver
transplantation
.
We
lack
accurate
diagnostic
methods
to
detect
premalignant
changes
of
the
biliary
epithelium
.
Diseases
Validation
Diseases presenting
"cholestasis"
symptom
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
congenital toxoplasmosis
erythropoietic protoporphyria
familial mediterranean fever
megacystis-microcolon-intestinal hypoperistalsis syndrome
neonatal adrenoleukodystrophy
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated