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Childhood mastocytosis.
[cutaneous mastocytosis]
Important
advances
have
been
achieved
in
recent
years
in
adult
mastocytosis
.
However
,
our
knowledge
about
childhood
mastocytosis
is
limited
because
invasive
tests
are
not
routinely
performed
in
children
.
We
ignore
the
frequency
of
systemic
involvement
in
childhood
mastocytosis
,
its
outcome
,
and
which
are
the
main
clinical
and
laboratory
parameters
associated
with
persistence
into
adult
mastocytosis
and
its
severity
.
Childhood
mastocytosis
is
a
clonal
mast
cell
disease
,
with
different
activating
mutations
in
the
KIT
gene
discovered
in
most
patients
.
Serum
tryptase
is
the
best
marker
for
mast
cell
burden
in
children
,
and
,
at
baseline
,
correlates
well
with
the
severity
of
symptoms
in
childhood
mastocytosis
.
Systemic
mastocytosis
definitely
may
occur
in
children
,
but
bone
marrow
studies
to
demonstrate
a
systemic
involvement
are
not
routinely
performed
nor
recommended
;
it
can
be
estimated
that
around
30
%
of
children
may
have
bone
marrow
involvement
as
demonstrated
by
showing
aggregates
of
mast
cells
or
by
flow
cytometry
of
mast
cells
expressing
the
aberrant
CD
2
5
marker
.
A
new
and
improved
classification
of
childhood
mastocytosis
is
needed
,
and
should
be
based
on
the
correlation
of
clinical
manifestations
,
morphology
of
mast
cells
in
the
skin
,
and
the
predicted
outcome
of
the
disease
.
The
current
classifications
of
childhood
mastocytosis
do
not
address
any
of
these
important
issues
.
Diseases
Validation
Diseases presenting
"its severity"
symptom
cutaneous mastocytosis
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