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A national survey of patients with intestinal motility disorders who are potential candidates for intestinal transplantation in Japan.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Intestinal
motility
disorders
are
a
major
cause
of
intestinal
failure
.
Severe
cases
such
as
idiopathic
pseudo-obstruction
represent
life-threatening
illnessed
.
Intestinal
transplantation
is
a
treatment
for
severe
motility
disorders
with
irreversible
intestinal
failure
.
However
,
the
prevalence
of
severe
motility
disorders
is
unknown
.
We
performed
a
national
survey
to
identify
patients
with
intestinal
motility
disorders
who
require
an
intestinal
transplant
.
The
national
survey
of
302
institutions
treating
intestinal
motility
disorders
identified
147
patients
treated
from
2006
to
2011
at
46
institutions
.
The
mean
patient
age
was
12
.
1
years
(
range
,
0
.
3
-
77
.
5
)
.
The
mean
age
of
onset
was
3
.
0
years
(
range
,
0
.
0
-
68
.
8
)
.
Diagnoses
included
chronic
idiopathic
intestinal
pseudo-obstruction
(
n
=
96
)
,
Hirschsprung
disease
(
n
=
29
)
,
megacystis
microcolon
intestinal
hypoperistalsis
syndrome
(
n
=
18
)
,
and
other
(
n
=
6
)
.
There
were
126
survivors
and
21
patients
who
died
during
the
last
5
years
.
The
mortality
rate
was
14
.
3
%
.
Eighty
-
five
percent
of
patients
required
parenteral
nutrition
for
more
than
6
months
,
which
was
defined
as
irreversible
intestinal
failure
.
Among
surviving
patients
with
irreversible
intestinal
failure
,
8
(
9
.
4
%
)
developed
hepatic
failure
with
jaundice
and
27
(
31
.
8
%
)
2
or
more
central
vein
thromboses
.
In
all
,
at
least
35
patients
(
41
%
)
with
irreversible
failure
due
to
intestinal
motility
disorders
may
be
candidates
for
transplantation
.
The
prevalence
of
severe
intestinal
motility
disorders
was
elucidated
in
Japan
.
Severe
cases
should
be
referred
to
transplant
centers
.