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Treatment of pruritus with Prometheus dialysis and absorption system in a patient with benign recurrent intrahepatic cholestasis.
[benign recurrent intrahepatic cholestasis]
Benign
recurrent
intrahepatic
cholestasis
(
BRIC
)
is
an
autosomal
recessive
liver
disorder
characterized
by
recurrent
episodes
of
jaundice
and
itching
.
Episodes
of
cholestasis
last
variously
from
1
week
to
several
months
,
may
start
at
any
age
and
usually
resolve
spontaneously
.
No
effective
treatment
has
been
found
as
yet
.
We
report
a
case
of
genetically
proven
BRIC
in
a
male
patient
who
developed
three
episodes
of
pruritus
and
jaundice
at
the
age
of
14
,
16
and
19
years
.
During
the
third
episode
,
he
did
not
respond
to
pharmacological
medical
therapy
,
and
fractionated
plasma
separation
and
absorption
(
FPSA
,
Prometheus
)
was
performed
to
manage
intractable
pruritus
.
The
treatment
immediately
alleviated
pruritus
,
lowered
serum
bilirubin
concentration
and
induced
sustained
remission
in
the
5
-
year
follow
up
.
FPSA
seems
to
be
a
safe
and
effective
way
of
treatment
for
BRIC
in
patients
with
severe
pruritus
and
prolonged
jaundice
.
