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Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome.
[megacystis-microcolon-intestinal hypoperistalsis syndrome]
Megacystis
microcolon
intestinal
hypoperistalsis
syndrome
(
MMIHS
)
is
a
rare
autosomal
recessive
disorder
causing
a
functional
neonatal
bowel
obstruction
.
Its
etiopathogenesis
is
not
fully
understood
.
The
prognosis
is
poor
in
the
majority
of
cases
;
most
patients
die
before
the
age
of
6
months
.
In
this
report
,
we
describe
our
experience
with
three
patients
with
MMIHS
in
whom
multivisceral
transplantation
was
performed
.
Three
patients
with
MMIHS
underwent
multivisceral
transplantation
.
All
patients
were
females
with
a
history
of
long
-term
total
parenteral
nutrition
(
TPN
)
with
TPN-related
cholestatic
liver
disease
.
Patient
1
died
17
months
after
transplantation
because
of
aspiration
after
revision
of
her
feeding
gastrostomy
.
At
the
time
of
death
,
the
graft
was
functioning
and
the
patient
was
completely
off
TPN
.
Patient
2
is
alive
17
months
after
transplant
.
She
is
a
fully
functional
,
active
2
-
year
-old
and
has
also
recently
begun
oral
feeding
after
intensive
rehabilitation
.
Patient
3
died
on
day
44
of
multisystem
failure
.
This
is
the
first
report
in
the
literature
of
multivisceral
transplantation
for
MMIHS
.
Although
one
of
the
three
patients
died
44
days
after
surgery
from
multiorgan
system
failure
,
the
other
two
patients
had
long
-term
survival
after
transplant
and
both
grew
well
on
enteral
feeding
alone
.
One
patient
died
17
months
from
a
non-transplant-related
complication
,
while
the
other
is
living
at
home
off
of
TPN
,
with
almost
complete
dietary
rehabilitation
17
months
after
transplant
.
Our
case
reports
suggest
that
multivisceral
transplantation
is
a
valuable
therapeutic
option
for
patients
affected
by
MMIHS
with
TPN-induced
liver
failure
.