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10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report.
[systemic capillary leak syndrome]
Idiopathic
systemic
capillary
leak
syndrome
(
ISCLS
)
is
a
rare
disorder
,
characterized
by
episodic
life-threatening
hypotension
,
hypoalbuminemia
,
and
hemoconcentration
.
A
10
-
year
-old
girl
presented
with
abdominal
pain
,
vomiting
,
diarrhea
,
fever
and
developed
generalized
edema
a
day
after
admission
.
Clinical
and
laboratory
findings
were
consistent
with
ISCLS
.
She
received
aggressive
fluid
replacement
,
methylprednisolone
pulse
(
30
mg
/
kg
/
day
)
,
high
-dose
intravenous
immunoglobulin
(
IVIG
,
2
g
/
kg
/
day
)
and
plasma
exchange
in
acute
phase
.
She
received
fasciotomy
of
bilateral
lower
extremities
as
she
developed
complications
of
compartment
syndrome
.
Since
there
were
two
episodes
of
ISCLS
attacks
,
theophylline
and
terbutaline
were
initiated
for
prevention
of
attacks
and
then
the
remission
is
currently
maintained
.
Because
of
high
fatality
rate
in
ISCLS
,
prompt
diagnosis
and
intervention
are
very
important
.
We
describe
here
,
a
rare
case
of
pediatric
ISCLS
.
ISCLS
should
be
considered
as
a
differential
diagnosis
,
when
the
patient
presents
with
unexplained
or
sudden
hypovolemic
shock
.
Reports
on
pediatrics
ISCLS
are
very
few
,
and
accumulation
of
similar
case
reports
is
needed
.
Diseases
Validation
Diseases presenting
"high fatality rate"
symptom
systemic capillary leak syndrome
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