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[Clarkson syndrome: a rare clinical condition characterized by generalized edema associated to monoclonal gammopathy].
[systemic capillary leak syndrome]
Generalized
edema
is
commonly
due
to
cardiac
failure
,
renal
changes
,
hepatic
and
metabolic
disturbances
,
or
it
can
be
idiopathic
,
i
.
e
.
primitive
lymphedema
.
Here
we
describe
a
patient
with
several
episodes
of
fluid
extravasation
characterized
by
hypotension
,
hemoconcentration
and
functional
renal
insufficiency
.
These
findings
,
associated
to
a
monoclonal
gammopathy
,
lead
to
the
diagnosis
of
systemic
capillary
leak
syndrome
or
Clarkson
Syndrome
.
This
rare
and
perplexing
disorder
,
characterized
by
a
typical
three
-phase
clinical
feature
,
is
due
to
an
endothelium
permeability
alteration
,
rather
responsible
of
these
paroxysmal
manifestations
.
Interleukin-
2
-
pathway
is
considered
as
one
of
the
underlying
mechanisms
.
During
acute
phase
the
patient
underwent
therapy
with
plasma-expanders
and
glucocorticoids
,
although
in
quiescent
phase
we
administered
aminophylline
,
salbutamol
and
prednisone
.
After
three
months
,
the
patient
is
asymptomatic
.
Diseases
Validation
Diseases presenting
"is due to an endothelium permeability alteration"
symptom
systemic capillary leak syndrome
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