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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
"recurrent episodes"
symptom
acute rheumatic fever
adrenomyeloneuropathy
benign recurrent intrahepatic cholestasis
child syndrome
cholangiocarcinoma
cystinuria
familial mediterranean fever
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
neuralgic amyotrophy
pyruvate dehydrogenase deficiency
systemic capillary leak syndrome
triple a syndrome
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