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Isolated liver and multivisceral transplantation for total parenteral nutrition-related end-stage liver disease.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Total
parenteral
nutrition
(
TPN
)
has
prolonged
survival
in
children
with
intestinal
failure
;
however
,
end-
stage
liver
disease
owing
to
TPN-induced
cholestasis
(
ESLD-TPN
)
may
preclude
its
use
.
ESLD-TPN
is
an
indication
for
isolated
liver
transplantation
(
ILT
)
or
multivisceral
transplantation
(
MVT
)
.
Isolated
liver
transplantation
for
ESLD-TPN
should
only
be
considered
in
patients
who
have
the
potential
for
enteral
autonomy
.
We
retrospectively
reviewed
the
records
of
patients
with
ESLD-TPN
who
underwent
ILT
(
n
=
7
)
or
MVT
(
n
=
5
)
between
1994
and
2005
.
The
median
age
at
the
time
of
transplantation
was
10
.
0
months
.
Intestinal
failure
followed
necrotizing
enterocolitis
(
n
=
3
)
,
gastroschisis
(
n
=
3
)
,
gastroschisis
with
volvulus
(
n
=
3
)
,
gastroschisis
with
atresia
(
n
=
1
)
,
malrotation
(
n
=
1
)
,
and
megacystis
microcolon
intestinal
hypoperistalsis
syndrome
(
n
=
1
)
.
Isolated
liver
transplant
patients
had
a
median
length
of
small
bowel
of
70
cm
and
tolerated
a
median
of
50
%
of
enteral
calories
.
The
median
length
of
small
bowel
in
patients
who
underwent
MVT
was
29
cm
,
and
none
tolerated
more
than
30
%
of
goal
enteral
feeds
.
Reduced-size
(
n
=
5
)
and
whole-
liver
(
n
=
2
)
allografts
were
used
for
patients
undergoing
ILT
.
Patients
undergoing
MVT
received
liver
-
small
bowel
-pancreas
(
n
=
4
)
or
liver
-
small
bowel
-pancreas-
colon
(
n
=
1
)
.
Overall
patient
survival
was
57
%
in
ILT
(
median
follow-up
=
25
.
1
months
)
;
3
survivors
are
TPN
independent
,
and
the
fourth
patient
requires
TPN
3
days
/
wk
.
Patient
survival
was
40
%
after
MVT
(
median
follow-up
=
13
.
0
months
)
;
1
MVT
patient
died
of
abuse
16
.
9
months
after
transplant
and
was
TPN
independent
at
the
time
of
death
.
Both
survivors
are
TPN
independent
.
Bilirubin
levels
are
within
normal
range
in
all
survivors
.
Isolated
liver
transplantation
for
ESLD-TPN
in
the
setting
of
intestinal
failure
is
a
viable
option
in
patients
who
have
the
potential
for
enteral
autonomy
.
Multivisceral
transplantation
is
the
only
alternative
in
patients
without
the
potential
for
intestinal
recovery
.
Survival
can
be
achieved
in
patients
with
ESLD-TPN
,
but
mortality
remains
high
for
both
procedures
.
Diseases
Validation
Diseases presenting
"stage liver disease"
symptom
benign recurrent intrahepatic cholestasis
erythropoietic protoporphyria
megacystis-microcolon-intestinal hypoperistalsis syndrome
primary hyperoxaluria type 1
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