Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome).
[systemic capillary leak syndrome]
The
systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
disorder
characterized
by
transient
episodes
of
hypotensive
shock
and
anasarca
thought
to
arise
from
reversible
microvascular
barrier
dysfunction
.
Although
the
high
prevalence
of
a
monoclonal
gammopathy
of
unknown
significance
in
SCLS
suggests
a
pathogenic
contribution
of
endogenous
immunoglobulins
,
the
mechanisms
of
vascular
hyperpermeability
remain
obscure
.
Herein
,
we
report
clinical
and
molecular
findings
on
23
patients
,
the
largest
SCLS
case
series
to
date
.
Application
of
episodic
SCLS
sera
,
but
neither
the
purified
immunoglobulin
fraction
nor
sera
obtained
from
patients
during
remission
,
to
human
microvascular
endothelial
cells
caused
vascular
endothelial
cadherin
internalization
,
disruption
of
interendothelial
junctions
,
actin
stress
fiber
formation
,
and
increased
permeability
in
complementary
functional
assays
without
inducing
endothelial
apoptosis
.
Intravenous
immunoglobulin
,
one
promising
therapy
for
SCLS
,
mitigated
the
permeability
effects
of
episodic
sera
.
Consistent
with
the
presence
of
endogenous
,
nonimmunoglobulin
,
circulating
permeability
factor
(
s
)
constrained
to
SCLS
episodes
,
we
found
that
vascular
endothelial
growth
factor
(
VEGF
)
and
angiopoietin
2
(
Ang
2
)
,
were
elevated
in
episodic
SCLS
sera
but
not
in
remission
sera
.
Ab
-based
inhibition
of
Ang
2
counteracted
permeability
induced
by
episodic
SCLS
sera
.
Comparable
experiments
with
anti-
VEGF
Ab
(
bevacizumab
)
yielded
less
interpretable
results
,
probably
because
of
endothelial
toxicity
of
VEGF
withdrawal
.
Our
results
support
a
model
of
SCLS
pathogenesis
in
which
nonimmunoglobulin
humoral
factors
such
as
VEGF
and
Ang
2
contribute
to
transient
endothelial
contraction
,
suggesting
a
molecular
mechanism
for
this
highly
lethal
disorder
.
Diseases
Validation
Diseases presenting
"vascular endothelial growth factor"
symptom
adrenal incidentaloma
aniridia
coats disease
dedifferentiated liposarcoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
inclusion body myositis
krabbe disease
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
severe combined immunodeficiency
systemic capillary leak syndrome
von hippel-lindau disease
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom