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Intractable cough and abnormal pulmonary function in benign recurrent intrahepatic cholestasis.
[benign recurrent intrahepatic cholestasis]
Benign
recurrent
intrahepatic
cholestasis
(
BRIC
)
is
a
syndrome
characterized
by
recurrent
episodes
of
cholestasis
with
associated
pruritus
.
The
intensity
and
duration
of
cholestatic
episodes
,
and
the
length
of
the
intervening
periods
,
vary
unpredictably
.
We
report
the
case
of
a
patient
with
BRIC
who
was
incapacitated
by
a
severe
intractable
cough
that
accompanied
marked
pruritus
during
her
second
cholestatic
episode
.
No
cause
for
the
cough
was
found
,
and
it
resolved
spontaneously
with
amelioration
of
mild
restrictive
abnormalities
of
pulmonary
function
as
the
cholestasis
subsided
.
Although
cough
has
not
been
recognized
as
a
complication
of
cholestasis
,
we
postulate
that
it
may
occur
either
(
i
)
as
a
result
of
direct
stimulation
of
sensory
nerves
by
circulating
humoral
substances
related
to
the
cholestasis
that
act
either
peripherally
in
the
airways
or
centrally
,
or
(
ii
)
through
stimulation
of
the
vagus
nerve
in
the
liver
,
leading
to
cough
that
is
mediated
either
centrally
or
by
reflex
.
Diseases
Validation
Diseases presenting
"intrahepatic cholestasis"
symptom
benign recurrent intrahepatic cholestasis
zellweger syndrome
This symptom has already been validated