Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis.
[systemic capillary leak syndrome]
Systemic
capillary
leak
syndrome
(
SCLS
)
is
a
rare
disorder
characterized
by
recurrent
spontaneous
episodes
of
hypovolaemic
shock
due
to
marked
plasma
shifts
from
the
intravascular
to
the
extravascular
space
.
This
presents
as
the
characteristic
triad
of
hypotension
,
haemoconcentration
and
hypoalbuminemia
often
with
an
associated
monoclonal
gammopathy
.
We
describe
a
patient
with
SCLS
who
required
aggressive
fluid
resuscitation
and
emergency
fasciotomies
for
compartment
syndrome
with
rhabdomyolysis
.
At
presentation
the
patient
was
considered
to
have
severe
erythrocytosis
and
was
therefore
initially
referred
to
a
haematologist
,
which
appears
to
be
a
frequent
sequence
of
presentation
for
patients
with
SCLS
.
This
patient
also
highlights
the
importance
of
muscle
compartment
pressure
monitoring
during
volume
resuscitation
in
patients
with
SCLS
.
Diseases
Validation
Diseases presenting
"hypotension"
symptom
achondroplasia
adrenal incidentaloma
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cutaneous mastocytosis
fabry disease
familial mediterranean fever
primary effusion lymphoma
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
typhoid
This symptom has already been validated