Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
[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
"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