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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
"hemoconcentration"
symptom
systemic capillary leak syndrome
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