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Clinical aspects of paediatric mastocytosis: a review of 101 cases.
[cutaneous mastocytosis]
Cutaneous
mastocytosis
(
CM
)
is
a
typical
presentation
of
mastocytosis
in
children
.
However
,
systemic
mastocytosis
may
also
occur
in
children
.
We
tried
to
characterize
the
clinical
features
of
childhood-onset
mastocytosis
and
estimate
the
value
of
the
SCORMA
(
SCORing
Mastocytosis
)
Index
and
serum
tryptase
levels
as
disease
severity
parameters
.
In
a
survey
of
101
children
mastocytosis
was
diagnosed
and
classified
according
to
World
Health
Organization
criteria
.
In
all
the
cases
serum
tryptase
levels
and
the
SCORMA
Index
were
done
to
assess
the
extent
and
intensity
of
the
disease
.
Cutaneous
mastocytosis
was
diagnosed
in
100
children
;
84
%
of
them
presented
maculopapular
CM
,
10
%
mastocytoma
and
6
%
diffuse
cutaneous
mastocytosis
.
Moreover
,
systemic
mastocytosis
with
bone
marrow
infiltration
and
associated
with
maculopapular
CM
was
found
in
one
case
.
There
was
a
positive
correlation
of
serum
tryptase
level
to
the
SCORMA
Index
.
Both
the
mean
tryptase
level
and
the
mean
SCORMA
Index
were
elevated
in
diffuse
cutaneous
mastocytosis
children
when
compared
with
other
forms
CM
.
A
significantly
higher
mean
tryptase
level
was
found
in
children
with
flushing
,
hypotension
,
diarrhoea
,
extensive
bullous
lesions
and
osteoporosis
or
osteopenia
.
Mastocytosis
in
children
usually
has
a
benign
course
.
Nevertheless
,
severe
mediator-related
symptoms
and
systemic
involvement
may
appear
.
Therefore
,
a
multidisciplinary
approach
involving
careful
monitoring
of
the
serum
tryptase
level
,
SCORMA
Index
and
the
organ
function
is
recommended
.
Both
tryptase
levels
and
the
SCORMA
Index
are
of
a
great
value
as
disease
severity
parameters
and
they
should
be
assessed
simultaneously
in
all
mastocytosis
patients
.
Diseases
Validation
Diseases presenting
"positive correlation"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
adrenal incidentaloma
aromatase deficiency
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cutaneous mastocytosis
dedifferentiated liposarcoma
familial mediterranean fever
hodgkin lymphoma, classical
inclusion body myositis
kallmann syndrome
legionellosis
oral submucous fibrosis
trochlear dysplasia
well-differentiated liposarcoma
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