Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience.
[systemic capillary leak syndrome]
To
determine
clinical
features
,
natural
history
,
and
outcome
of
a
well-defined
cohort
of
25
consecutive
patients
with
idiopathic
systemic
capillary
leak
syndrome
(
SCLS
)
evaluated
at
a
tertiary
care
center
.
Records
of
patients
diagnosed
as
having
SCLS
from
November
1
,
1981
,
through
April
30
,
2008
,
were
reviewed
.
Descriptive
statistics
were
used
to
analyze
patient
demographics
,
clinical
features
,
complications
,
and
therapeutic
interventions
.
Of
the
34
patients
whose
records
were
reviewed
,
25
fulfilled
all
diagnostic
criteria
for
SCLS
.
The
median
age
at
diagnosis
of
SCLS
was
44
years
.
Median
follow-up
of
surviving
patients
was
4
.
9
years
,
and
median
time
to
diagnosis
from
symptom
onset
was
1
.
1
years
(
interquartile
range
,
0
.
5
-
4
.
1
years
)
.
Flulike
illness
or
myalgia
was
reported
by
14
patients
(
56
%
)
at
onset
of
an
acute
attack
of
SCLS
,
and
rhabdomyolysis
developed
in
9
patients
(
36
%
)
.
Patients
with
a
greater
decrease
in
albumin
level
had
a
higher
likelihood
of
developing
rhabdomyolysis
(
p
=
.
03
)
.
Monoclonal
gammopathy
,
predominantly
of
the
IgG-κ
type
,
was
found
in
19
patients
(
76
%
)
.
The
progression
rate
to
multiple
myeloma
was
0
.
7
%
per
person-
year
of
follow-up
.
The
overall
response
rate
to
the
different
therapies
was
76
%
,
and
24
%
of
patients
sustained
durable
(
>
2
years
)
complete
remission
.
The
estimated
5
-
year
overall
survival
rate
was
76
%
(
95
%
confidence
interval
,
59
%
-
97
%
)
.
Systemic
capillary
leak
syndrome
,
a
rare
disease
that
occurs
in
those
of
middle
age
,
is
usually
diagnosed
after
a
considerable
delay
from
onset
of
symptoms
.
The
degree
of
albumin
decrement
during
an
attack
correlates
with
development
of
rhabdomyolysis
.
A
reduction
in
the
frequency
and
/
or
the
severity
of
attacks
was
seen
in
nearly
three
-fourths
of
patients
who
were
offered
empirical
therapies
.
The
rate
of
progression
to
multiple
myeloma
appears
to
be
comparable
to
that
of
monoclonal
gammopathy
of
undetermined
significance
.
Diseases
Validation
Diseases presenting
"myalgia"
symptom
familial mediterranean fever
focal myositis
pendred syndrome
pyomyositis
scrub typhus
systemic capillary leak syndrome
This symptom has already been validated
