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Systemic capillary leak syndrome. Case report.
[systemic capillary leak syndrome]
We
here
report
the
case
of
a
patient
with
systemic
capillary
leak
syndrome
(
SCLS
)
.
This
syndrome
is
a
rare
condition
characterized
by
recurrent
episodes
of
hypotension
with
hemoconcentration
and
hypoproteinemia
.
It
is
due
to
unexplained
episodic
capillary
hyperpermeabilty
that
results
in
fluid
and
protein
shift
from
the
intravascular
to
the
interstitial
space
:
generalized
edema
,
shock
and
renal
failure
follow
.
A
59
yo
man
was
admitted
to
our
intensive
care
unit
because
of
unexplained
shock
with
hemoconcentration
,
renal
failure
,
and
metabolic
acidosis
.
Previous
attemps
to
reverse
shock
in
a
medical
ward
with
crystalloids
and
dopamine
failed
.
An
abdominal
CT
scan
,
a
TEE
,
and
chest
X
ray
study
were
inconclusive
.
No
sign
or
history
of
major
infections
or
anaphylaxis
were
present
.
The
patient
was
resuscitated
with
massive
fluid
infusions
and
norepinephrine
on
the
guide
of
a
Swan
Ganz
catheter
.
The
diagnosis
was
made
on
the
basis
of
a
previous
episode
of
severe
shock
complicated
with
renal
failure
and
a
compartment
syndrome
,
the
hemoconcentration
,
and
the
negative
cardiopulmonary
findings
.
A
small
amount
of
monoclonal
immunoglobulin
G
,
kappa
chain
,
found
in
the
serum
confirmed
the
diagnosis
.
The
SCLS
should
be
considered
in
the
differential
diagnosis
of
idiopathic
and
anaphylactic
shock
.
Patients
may
benefit
from
a
prophylactic
treatment
with
theophilline
and
terbutaline
.
Diseases
Validation
Diseases presenting
"renal failure"
symptom
cholangiocarcinoma
congenital diaphragmatic hernia
cystinuria
erdheim-chester disease
fabry disease
heparin-induced thrombocytopenia
homocystinuria without methylmalonic aciduria
junctional epidermolysis bullosa
kindler syndrome
legionellosis
oculocutaneous albinism
primary hyperoxaluria type 1
scrub typhus
systemic capillary leak syndrome
This symptom has already been validated