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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
"diarrhea"
symptom
acute rheumatic fever
adrenomyeloneuropathy
benign recurrent intrahepatic cholestasis
cutaneous mastocytosis
esophageal carcinoma
fabry disease
familial mediterranean fever
hirschsprung disease
kabuki syndrome
legionellosis
lymphangioleiomyomatosis
omenn syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
scrub typhus
systemic capillary leak syndrome
triple a syndrome
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
wiskott-aldrich syndrome
This symptom has already been validated