Rare Diseases Symptoms Automatic Extraction
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Initial evidence of endothelial cell apoptosis as a mechanism of systemic capillary leak syndrome.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
disorder
of
unknown
etiology
that
is
characterized
by
acute
recurrent
attacks
of
hypovolemic
shock
commonly
following
an
inflammatory
stimulus
such
as
a
viral
illness
.
Prophylactic
therapy
is
generally
ineffective
,
and
the
outcome
is
frequently
fatal
.
In
order
to
investigate
the
cellular
mechanisms
leading
to
SCLS
,
we
examined
the
effects
of
sera
from
two
patients
with
active
SCLS
on
microvascular
endothelial
cell
apoptosis
in
vitro
.
Apoptosis
was
determined
by
morphologic
criteria
,
DNA
fragmentation
,
annexin
V
stain
,
and
by
a
quantitative
photometric
assay
.
The
apoptotic
pathway
was
investigated
by
Western
blot
of
endothelial
cells
lysate
after
exposure
to
SCLS
sera
.
The
sera
from
patients
with
active
SCLS
mediated
profound
apoptosis
of
microvascular
endothelial
cells
shortly
after
exposure
.
The
exposed
microvascular
endothelial
cells
underwent
immediate
apoptosis
as
evidenced
by
morphologic
changes
,
plasma
membrane
phosphatidylserine
exposure
,
and
by
DNA
fragmentation
.
Increased
Bax
/
Bcl-
2
ratio
in
endothelial
cells
exposed
to
SCLS
sera
was
observed
and
suggested
an
oxidation
injury
as
the
possible
mechanism
for
endothelial
apoptosis
.
This
potential
mechanism
was
further
explored
by
measuring
intracellular
reactive
oxygen
species
(
ROS
)
following
SCLS
serum
exposure
.
Sera
from
both
patients
caused
marked
increases
in
ROS
,
initially
detectable
at
1
h
and
persisted
for
at
least
12
h
,
with
control
serum
from
healthy
subjects
showing
no
effect
on
basal
endothelial
cell
ROS
concentrations
.
Components
from
the
sera
of
patients
with
active
systemic
capillary
leak
syndrome
in
contrast
to
healthy
subject
sera
mediate
early
and
extensive
endothelial
apoptosis
in
vitro
that
is
associated
with
oxidation
injury
.
These
data
represent
compelling
initial
evidence
for
oxidation-induced
apoptosis
as
a
likely
mechanism
for
endothelial
injury
leading
to
SCLS
.