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Cholestatic liver diseases: slow progress in understanding and treating slowly progressive disorders.
[benign recurrent intrahepatic cholestasis]
Cholestatic
liver
diseases
are
characterized
by
failure
of
normal
amounts
of
physiological
bile
to
reach
the
gastrointestinal
tract
.
Any
interference
with
normal
bile
flow
from
the
canalicular
membrane
of
the
hepatocyte
to
the
distal
common
bile
duct
may
result
in
cholestasis
.
Literature
review
.
In
primary
biliary
cirrhosis
(
PBC
)
,
the
small
intrahepatic
bile
ducts
are
destructed
,
resulting
in
obstruction
of
intrahepatic
bile
flow
,
whereas
extrahepatic
and
/
or
intrahepatic
biliary
strictures
block
the
passage
of
bile
towards
the
intestine
in
primary
sclerosing
cholangitis
(
PSC
)
.
In
contrast
,
the
biliary
tree
is
morphologically
unaffected
in
less
common
cholestatic
liver
diseases
as
benign
recurrent
intrahepatic
cholestasis
(
BRIC
)
and
progressive
familiar
intrahepatic
cholestasis
(
PFIC
1
-
4
)
.
Genetic
defects
in
hepatic
canalicular
transport
mechanisms
and
bile
salt
synthesis
deficiencies
seem
to
underlie
these
types
of
cholestatic
disorders
.
Recent
advances
in
understanding
and
treatment
of
cholestatic
liver
diseases
may
help
in
better
diagnosing
and
treating
the
various
conditions
characterized
by
cholestasis
.
