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A novel in vivo model of permanent intestinal aganglionosis.
[hirschsprung disease]
Enteric
neuromuscular
disease
is
a
characteristic
of
several
disease
states
,
including
Hirschsprung
disease
,
esophageal
achalasia
,
Chagas
disease
,
and
gastroparesis
.
Medical
therapy
for
these
conditions
is
limited
,
and
surgical
intervention
may
incur
significant
morbidity
.
Alternatively
,
transplantation
of
neural
progenitor
cells
may
regenerate
enteric
ganglia
.
Existing
aganglionosis
model
systems
are
limited
by
swift
animal
demise
or
by
spontaneous
regeneration
of
native
ganglia
.
We
propose
a
novel
protocol
to
induce
permanent
aganglionosis
in
a
segment
of
rat
jejunum
,
which
may
serve
as
an
experimental
transplantation
target
for
cellular
therapy
.
This
protocol
was
performed
in
17
adult
female
Sprague-
Dawley
rats
.
A
laparotomy
was
performed
and
a
1
-
cm
segment
of
jejunum
was
isolated
from
continuity
.
Among
14
rats
,
the
isolated
segments
were
treated
with
benzalkonium
chloride
(
BAC
)
for
20
min
to
induce
aganglionosis
.
Jejunal
segment
isolation
was
performed
without
BAC
treatment
in
three
rats
.
The
animals
were
euthanized
at
posttreatment
days
21
-
166
.
Muscle
layer
diameter
was
compared
among
normal
,
isolated
,
and
BAC-treated
isolated
jejunal
segments
.
The
presence
of
jejunal
ganglia
was
documented
by
immunohistochemical
staining
(
IHC
)
for
beta
-
III
tubulin
(
TUJ
1
)
and
S
100
,
markers
of
neuronal
and
glial
cell
lineages
,
respectively
.
Ganglia
were
identified
by
IHC
in
normal
and
isolated
jejunal
segments
.
Isolated
segments
had
significantly
hypertrophied
smooth
muscle
layers
compared
with
normal
jejunum
(
diameter
343
±
53
μm
versus
211
±
37
μm
,
P
<
0
.
0001
)
.
BAC-treated
jejunal
segments
had
no
IHC
evidence
of
ganglionic
structures
.
Aganglionosis
was
persistent
in
all
specimens
up
to
166
days
after
treatment
.
The
exclusion
of
a
jejunal
segment
from
continuity
and
concurrent
treatment
with
BAC
results
in
an
effective
,
reproducible
,
and
permanent
model
of
aganglionosis
.
Muscular
hypertrophy
and
aganglionosis
in
the
isolated
jejunal
segment
make
it
an
ideal
recipient
site
for
transplantation
of
neuroglial
precursor
cells
.
Diseases
Validation
Diseases presenting
"isolated jejunal segments. isolated segments"
symptom
hirschsprung disease
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