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Benign recurrent intrahepatic cholestasis: a long-term follow-up.
[benign recurrent intrahepatic cholestasis]
We
report
on
a
case
with
a
50
-
year
history
of
benign
recurrent
intrahepatic
cholestasis
,
including
33
years
of
follow-up
at
our
hospital
.
The
attacks
of
jaundice
began
at
the
age
of
1
year
and
have
been
repeated
more
than
14
times
to
date
.
During
the
follow-up
period
at
our
hospital
,
the
patient
has
suffered
6
attacks
of
cholestasis
.
During
the
episodes
of
cholestasis
,
a
dissociation
between
the
serum
level
of
bilirubin
and
that
of
bile
acids
was
observed
.
The
analysis
of
serum
bile
acids
during
cholestasis
showed
that
cholic
acid
and
chenodeoxycholic
acid
were
the
main
bile
acids
,
and
no
appreciable
amounts
of
the
secondary
bile
acids
were
detected
.
Between
episodes
the
liver
function
tests
,
including
serum
bile
acids
,
were
normal
.
Despite
multiple
attacks
of
cholestasis
over
many
years
,
no
permanent
liver
damage
has
occurred
.
This
case
reaffirms
the
essentially
benign
nature
of
this
disease
.
Diseases
Validation
Diseases presenting
"jaundice"
symptom
alpha-thalassemia
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
cholangiocarcinoma
congenital toxoplasmosis
erythropoietic protoporphyria
esophageal carcinoma
homocystinuria without methylmalonic aciduria
megacystis-microcolon-intestinal hypoperistalsis syndrome
neonatal adrenoleukodystrophy
oculocutaneous albinism
phenylketonuria
scrub typhus
typhoid
von hippel-lindau disease
This symptom has already been validated