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Control of systemic capillary leak syndrome with aminophylline and terbutaline.
[systemic capillary leak syndrome]
Patients
with
systemic
capillary
leak
syndrome
have
a
characteristic
triad
of
hypotension
,
hemoconcentration
,
and
monoclonal
gammopathy
.
They
have
frequent
and
severe
attacks
of
hemoconcentration
and
hypotension
accompanied
by
marked
plasma
shifts
.
The
exact
role
of
this
monoclonal
protein
is
unknown
,
but
it
probably
leads
,
in
some
way
,
to
an
increase
in
capillary
permeability
.
Despite
efforts
to
resuscitate
the
patients
during
an
acute
attack
,
the
syndrome
is
often
fatal
.
Some
success
has
been
obtained
in
preventing
the
attacks
with
the
beta
-adrenergic-stimulating
agent
terbutaline
.
The
purpose
of
this
study
was
to
determine
the
effectiveness
of
aminophylline
and
terbutaline
in
the
treatment
of
systemic
capillary
leak
syndrome
.
Over
a
decade
,
three
patients
with
systemic
capillary
leak
syndrome
presented
at
our
institution
.
All
three
patients
were
treated
with
terbutaline
and
aminophylline
.
Prednisone
was
used
during
the
course
of
treatment
in
each
of
the
three
patients
.
In
contrast
to
previous
reports
of
partial
or
temporary
control
of
episodes
,
all
three
patients
are
alive
with
almost
complete
resolution
of
their
recurrent
attacks
and
have
been
able
to
return
to
their
normal
lifestyles
.
The
regimen
of
terbutaline
and
aminophylline
effectively
prevents
the
attacks
of
hypotension
and
hemoconcentration
that
occur
in
systemic
capillary
leak
syndrome
.
The
role
of
prednisone
is
not
clear
.
Until
more
is
known
about
the
pathophysiology
of
the
disorder
,
treatment
must
remain
empiric
and
supportive
.
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