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[Systemic capillary leak syndrome].
[systemic capillary leak syndrome]
A
man
aged
61
had
recurrent
attacks
of
severe
shock
.
The
episodes
were
preceded
by
symptoms
such
as
a
runny
nose
,
epigastric
discomfort
with
nausea
,
vertigo
,
orthostatism
and
sometimes
light
fever
.
During
the
attacks
there
were
marked
hypotension
,
a
strong
rise
of
the
haematocrit
,
a
decrease
of
the
protein
and
albumin
concentrations
in
the
blood
and
prerenal
kidney
failure
.
In
addition
,
there
was
a
paraprotein
,
type
IgG-kappa
.
The
shock
every
time
responded
rapidly
to
intravenous
administration
of
fluid
and
was
followed
by
a
period
of
substantial
polyuria
.
The
pattern
was
characteristic
of
systemic
capillary
leak
syndrome
,
a
rare
but
frequently
fatal
disease
characterized
by
episodes
of
unexplained
extravasation
of
plasma
.
The
aetiology
and
pathogenesis
are
unknown
.
Attacks
are
suppressed
by
supportive
therapy
(
administration
of
fluids
,
inotropics
)
and
future
attacks
may
be
prevented
by
the
intake
of
terbutaline
and
theophylline
.
The
systemic
capillary
leak
syndrome
should
be
considered
in
the
differential
diagnosis
of
idiopathic
and
anaphylactic
shock
.
Diseases
Validation
Diseases presenting
"fever"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
alexander disease
allergic bronchopulmonary aspergillosis
canavan disease
carcinoma of the gallbladder
child syndrome
congenital toxoplasmosis
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial mediterranean fever
focal myositis
hodgkin lymphoma, classical
lamellar ichthyosis
legionellosis
locked-in syndrome
malignant atrophic papulosis
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
papillon-lefèvre syndrome
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
triple a syndrome
typhoid
waldenström macroglobulinemia
wolf-hirschhorn syndrome
This symptom has already been validated
