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[The megacystis-microcolon-intestinal hypoperistalsis syndrome: apropos of a case with prolonged survival].
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis
microcolon
intestinal
hypoperistalsis
syndrome
(
MMIHS
)
is
a
severe
congenital
disease
with
autosomal
recessive
inheritance
,
characterized
by
vesical
distension
and
intestinal
hypoperistalsis
what
causes
intestinal
obstruction
in
newborn
,
with
other
abnormalities
associated
.
It
presents
a
low
incidence
,
about
a
hundred
cases
are
reported
in
the
bibliography
.
Life
expectancy
does
n't
reach
a
year
because
of
the
sepsis
failure
generally
.
In
our
study
the
survival
is
higher
than
the
majority
of
the
cases
reported
,
with
good
cuality
of
life
and
acceptable
ponderal
development
.
Home
parenteral
nutrition
with
the
following
and
multidisciplinary
collaboration
in
a
strict
way
,
establish
the
success
'
key
in
this
pathology
.