Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
,
non-
Langerhans
histiocytosis
.
Recent
findings
suggest
that
ECD
is
a
clonal
disorder
,
marked
by
recurrent
BRAFV
600
E
mutations
in
>
50
%
of
patients
,
in
which
chronic
uncontrolled
inflammation
is
an
important
mediator
of
disease
pathogenesis
.
Although
∼
500
to
550
cases
have
been
described
in
the
literature
to
date
,
increased
physician
awareness
has
driven
a
dramatic
increase
in
ECD
diagnoses
over
the
last
decade
.
ECD
frequently
involves
multiple
organ
systems
and
has
historically
lacked
effective
therapies
.
Given
the
protean
clinical
manifestations
and
the
lack
of
a
consensus-derived
approach
for
the
management
of
ECD
,
we
provide
here
the
first
multidisciplinary
consensus
guidelines
for
the
clinical
management
of
ECD
.
These
recommendations
were
outlined
at
the
First
International
Medical
Symposium
for
ECD
,
comprised
of
a
comprehensive
group
of
international
academicians
with
expertise
in
the
pathophysiology
and
therapy
of
ECD
.
Detailed
recommendations
on
the
initial
clinical
,
laboratory
,
and
radiographic
assessment
of
ECD
patients
are
presented
in
addition
to
treatment
recommendations
based
on
critical
appraisal
of
the
literature
and
clinical
experience
.
These
formalized
consensus
descriptions
will
hopefully
facilitate
ongoing
and
future
research
efforts
in
this
disorder
.
Diseases
Validation
Diseases presenting
"first multidisciplinary consensus guidelines for the clinical management"
symptom
erdheim-chester disease
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom