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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
.
Diseases
Validation
Diseases presenting
"severe cases"
symptom
coats disease
congenital diaphragmatic hernia
dentin dysplasia
dentinogenesis imperfecta
erythropoietic protoporphyria
hereditary cerebral hemorrhage with amyloidosis
hydrocephalus with stenosis of the aqueduct of sylvius
locked-in syndrome
megacystis-microcolon-intestinal hypoperistalsis syndrome
neuralgic amyotrophy
proteus syndrome
scrub typhus
trochlear dysplasia
typhoid
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