Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature.
[systemic capillary leak syndrome]
The
systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
condition
characterized
by
unexplained
episodic
attacks
of
systemic
capillary
hyperpermeability
accompanied
by
hypoalbuminemia
,
hemoconcentration
and
edema
.
Treatment
of
the
acute
phase
is
supportive
,
focusing
on
adequate
fluid
resuscitation
.
Many
agents
have
been
used
to
prevent
acute
attacks
,
including
corticosteroids
,
β
2
-
agonists
(
aminophylline
,
theophylline
,
or
terbutaline
)
,
infliximab
,
thalidomide
and
intravenous
immunoglobulin
(
IVIg
)
.
β
2
-
agonists
were
the
first
-line
maintenance
therapy
until
a
few
years
ago
.
In
more
recent
years
,
IVIg
became
common
first
-line
prophylactic
therapy
in
most
patients
with
benefits
at
the
dose
of
2
g
/
kg
once
a
month
.
We
report
the
case
of
a
49
-
year
-old
man
with
SCLS
treated
successfully
with
a
lower
dose
of
IVIg
(
1
g
/
kg
monthly
)
in
the
maintenance
phase
.
He
presented
no
acute
episodes
in
a
follow-up
of
28
months
.
We
describe
prophylactic
treatments
for
SCLS
in
literature
and
compare
our
patient
to
another
18
who
received
IVIg
in
follow-up
.
Diseases
Validation
Diseases presenting
"acute attacks"
symptom
erythropoietic protoporphyria
familial mediterranean fever
systemic capillary leak syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom