Rare Diseases Symptoms Automatic Extraction
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Erdheim-Chester disease in a child.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
systemic
non-
Langerhans
histiocytosis
that
affects
multiple
organ
systems
.
It
occurs
more
often
in
adults
,
and
paediatric
ECD
is
extremely
rare
.
The
diagnosis
of
ECD
can
be
established
based
on
clinical
presentations
and
imaging
but
the
final
diagnosis
should
be
based
on
biopsy
.
Treatment
of
ECD
has
involved
the
use
of
corticosteroids
,
radiotherapy
,
chemotherapy
,
surgery
and
haematopoietic
stem
cell
transplantation
,
yet
the
efficacy
of
these
treatments
is
difficult
to
determine
.
At
present
,
it
is
thought
that
the
treatment
of
interferon-alpha
(
IFN-alpha
)
is
safe
and
effective
for
ECD
.
Herein
,
we
report
on
an
11
-
year
old
girl
who
was
admitted
to
hospital
because
of
systemic
bone
pain
and
limping
,
and
the
final
diagnosis
of
ECD
was
based
on
evidence
provided
by
her
clinical
presentation
,
imaging
and
biopsy
of
a
lesion
of
the
right
ilium
.
The
patient
was
treated
with
subcutaneous
IFN-alpha
at
a
dosage
of
3
x
10
(
6
)
units
three
times
weekly
for
19
months
.
We
thought
that
the
treatment
of
IFN-alpha
was
safe
and
effective
for
the
girl
's
clinical
manifestations
,
and
IFN-alpha
might
be
a
valuable
first
-line
therapy
for
paediatric
ECD
.
Diseases
Validation
Diseases presenting
"bone pain"
symptom
aromatase deficiency
cutaneous mastocytosis
erdheim-chester disease
primary hyperoxaluria type 1
This symptom has already been validated