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Erdheim-Chester disease: an uncommon cause of upper urinary tract obstruction.
[erdheim-chester disease]
Erdheim-
Chester
disease
is
a
rare
non-
Langerhans
form
of
systemic
histiocytosis
of
unknown
origin
.
We
describe
a
45
-
year
-old
man
presenting
with
bilateral
hydronephrosis
suggestive
of
extrinsic
urinary
tract
obstruction
.
Computed
tomography
revealed
extensive
hypodense
soft
tissue
infiltration
in
the
retroperitoneum
surrounding
the
kidneys
.
Needle
biopsy
of
the
retroperitoneal
soft
tissue
revealed
aggregates
of
lipid-laden
histiocytes
expressing
CD
68
but
negative
for
CD
1
a
and
S
100
protein
.
The
diagnosis
of
Erdheim-
Chester
disease
was
supported
by
typical
radionuclide
bone
scinitigraphic
findings
.
Treatment
with
prednisolone
,
sirolimus
,
and
regular
ureteric
stent
revision
was
initiated
to
achieve
adequate
urinary
tract
drainage
.
To
our
knowledge
,
this
is
the
second
patient
with
Erdheim-
Chester
disease
reported
in
Hong
Kong
.
A
high
index
of
suspicion
is
required
to
avoid
delay
in
the
diagnosis
of
this
rare
disease
.
Diseases
Validation
Diseases presenting
"adequate urinary tract drainage"
symptom
erdheim-chester disease
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