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[Complexity of pathological interpretation in megacystis-microcolon-intestinal hypoperistalsis syndrome].
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis-
microcolon
-
intestinal
hypoperistalsis
syndrome
is
very
rare
,
and
is
the
most
severe
of
the
chronic
intestinal
pseudoobstructions
.
Diagnosis
is
usually
made
in
the
neonatal
period
,
is
clinical
and
radiological
,
and
is
confirmed
by
manometric
studies
.
Microscopic
abnormalities
are
variable
,
inconstant
and
nonspecific
.
They
involve
the
smooth
muscle
more
often
than
the
intrinsic
innervation
of
the
gut
and
the
bladder
.
A
girl
,
currently
seven
years
old
,
presented
with
megacystis
observed
on
prenatal
ultrasound
at
21
weeks
of
gestation
.
At
first
,
amniotic
fluid
volume
was
appropriate
for
gestational
age
,
and
then
hydramnios
appeared
at
30
weeks
of
gestation
.
Microcolon
was
discovered
at
birth
,
with
microileum
,
dilatation
of
the
duodenum
and
proximal
jejunum
,
intestinal
malposition
,
and
severe
hypoperistalsis
of
the
entire
gastrointestinal
tract
,
which
indicated
enterostomy
and
total
parenteral
nutrition
from
birth
.
At
pathological
examination
,
rectal
biopsy
and
enteric
nervous
plexuses
were
normal
.
There
was
hypoplasia
of
the
external
longitudinal
layer
of
the
muscularis
propria
in
the
colon
and
ileum
.
Cajal
cells
could
not
be
demonstrated
immunohistochemically
in
the
colon
.
This
case
highlights
the
complexity
and
difficulties
of
pathological
interpretation
in
this
syndrome
,
and
the
necessity
of
a
large
study
of
controls
at
different
ages
and
different
levels
of
the
digestive
tract
and
the
bladder
.
Diseases
Validation
Diseases presenting
"and the necessity of a large study of controls at different ages and different levels of the digestive tract and the bladder"
symptom
megacystis-microcolon-intestinal hypoperistalsis syndrome
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