Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis.
[cutaneous mastocytosis]
Despite
the
good
prognosis
of
pediatric
mastocytosis
,
some
patients
suffer
from
severe
mast
cell
(
MC
)
mediator-associated
symptoms
.
The
aim
of
this
study
was
to
identify
predictors
for
severe
MC
mediator
release
symptoms
in
children
with
mastocytosis
in
the
skin
(
MIS
)
.
Serum
baseline
total
tryptase
(
sbT
)
levels
in
111
children
with
MIS
-
80
maculopapular
cutaneous
mastocytosis
/
plaque
mastocytosis
,
22
nodular
mastocytosis
,
and
nine
diffuse
cutaneous
mastocytosis
-
were
investigated
as
a
predictive
biomarker
for
the
occurrence
of
MC
mediator-related
signs
and
symptoms
within
the
first
18
months
after
disease
onset
.
Twelve
children
(
11
%
)
who
showed
extensive
cutaneous
disease
involving
>
90
%
of
body
surface
area
(
BSA
)
suffered
from
severe
symptoms
requiring
hospitalization
,
with
(
n
=
5
)
or
without
(
n
=
6
)
management
in
the
intensive
care
unit
(
ICU
)
owing
to
life-threatening
complications
.
The
median
sbT
was
significantly
(
P
<
0
.
001
)
higher
in
patients
with
extensive
cutaneous
disease
vs
those
with
<
90
%
of
BSA
involved
(
45
.
5
vs
5
.
2
μg
/
l
,
respectively
)
,
as
well
as
in
children
with
grade
4
(
severe
mastocytosis
-related
symptoms
requiring
emergency
therapy
and
hospitalization
)
vs
those
with
grade
<
4
(
46
.
2
vs
5
.
2
μg
/
l
,
respectively
)
.
Receiver
operating
characteristics
curve
analyses
showed
that
the
optimal
cutoff
s
for
sbT
to
predict
the
need
for
daily
antimediator
therapy
,
hospitalization
,
and
the
management
in
an
ICU
were
6
.
6
,
15
.
5
,
and
30
.
8
μg
/
l
,
respectively
(
sensitivity
and
specificity
of
77
%
and
79
%
,
100
%
and
95
%
,
and
100
%
and
96
%
,
respectively
)
.
Increased
sbT
in
association
with
extensive
cutaneous
involvement
identifies
patients
at
risk
for
severe
MC
activation
events
in
pediatric
mastocytosis
.
Diseases
Validation
Diseases presenting
"severe mastocytosis-related symptoms"
symptom
cutaneous mastocytosis
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom