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Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Severe
gastrointestinal
dysmotility
(
GID
)
impairs
patients
'
quality
of
life
and
is
almost
uniformly
fatal
after
complications
of
parenteral
nutrition
.
Intestinal
and
multivisceral
transplants
have
been
used
as
alternative
treatment
of
these
disorders
.
We
studied
patients
with
GID
treated
with
transplantation
in
our
center
,
and
reviewed
their
outcome
to
determine
the
therapeutic
efficacy
of
multivisceral
transplants
.
T
he
transplant
database
was
searched
for
patients
with
GID
from
1994
to
2001
.
We
excluded
patients
with
Hirschsprung
disease
,
scleroderma
,
and
diabetic
enteropathy
.
We
reviewed
explanted
organs
,
histochemistry
,
and
immunohistochemistry
and
classified
cases
by
etiology
.
We
selected
12
children
with
GID
from
124
patients
transplanted
.
Nine
presented
before
1
year
and
3
started
with
symptoms
between
2
and
8
years
.
By
combined
clinical
and
histopathological
features
,
6
were
classified
as
megacystis
microcolon
intestinal
hypoperistalsis
syndrome
,
4
as
chronic
idiopathic
intestinal
pseudoobstruction
,
and
2
as
intestinal
neuronal
dysplasias
.
Six
patients
died
during
the
follow-up
from
21
to
546
days
after
transplant
.
The
Kaplan-
Meier
actuarial
survival
rates
were
66
.
7
%
at
1
year
and
50
%
at
3
years
.
Multivisceral
transplantation
is
a
valuable
therapeutic
alternative
for
children
with
severe
GID
who
can
not
be
adequately
managed
with
parenteral
nutrition
.
Diseases
Validation
Diseases presenting
"parenteral nutrition"
symptom
megacystis-microcolon-intestinal hypoperistalsis syndrome
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