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Benign recurrent intrahepatic cholestasis with secondary renal impairment treated with extracorporeal albumin dialysis.
[benign recurrent intrahepatic cholestasis]
Benign
recurrent
intrahepatic
cholestasis
(
BRIC
)
is
a
rare
autosomal
recessive
condition
characterized
by
intermittent
episodes
of
pruritus
and
jaundice
that
may
last
days
to
months
.
Treatment
is
often
ineffective
and
symptoms
,
particularly
pruritus
,
can
be
severe
.
Extracorporeal
albumin
dialysis
(
molecular
adsorbent
recycling
system
,
MARS
)
is
a
novel
treatment
which
removes
albumin
bound
toxins
including
bilirubin
and
bile
salts
.
We
describe
a
case
of
a
34
-
year
-old
man
with
BRIC
and
secondary
renal
impairment
who
,
having
failed
standard
medical
therapy
,
was
treated
with
MARS
.
The
treatment
immediately
improved
his
symptoms
,
renal
and
liver
function
tests
and
appeared
to
terminate
the
episode
of
cholestasis
.
We
conclude
that
MARS
is
a
safe
and
effective
treatment
for
BRIC
with
associated
renal
impairment
.
Diseases
Validation
Diseases presenting
"secondary renal impairment"
symptom
benign recurrent intrahepatic cholestasis
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