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Urologic manifestations of Erdheim-Chester disease.
[erdheim-chester disease]
To
describe
the
urologic
manifestations
of
Erdheim-
Chester
disease
(
ECD
)
.
ECD
is
a
rare
multisystem
disorder
of
non-
Langerhans
cell
histiocytosis
.
In
addition
to
classic
long
bone
involvement
,
the
retroperitoneum
is
a
well-established
site
of
disease
infiltration
.
Herein
,
we
present
the
urologic
manifestations
and
outcomes
of
ECD
in
a
large
series
of
patients
.
We
identified
47
patients
diagnosed
with
ECD
between
1996
and
2012
at
our
institution
.
The
medical
records
and
imaging
for
these
patients
were
reviewed
for
urologic
involvement
,
including
perirenal
soft
tissue
encasement
,
renal
atrophy
,
hydronephrosis
,
chronic
renal
insufficiency
,
diabetes
insipidus
,
and
lower
urinary
tract
symptoms
.
At
diagnosis
,
the
median
patient
age
was
57
years
(
interquartile
range
49
,
68
)
,
and
median
follow-up
after
diagnosis
was
3
years
(
interquartile
range
1
.
8
,
7
.
3
)
.
There
were
31
male
patients
(
66
%
)
and
16
female
patients
(
34
%
)
.
The
majority
of
these
patients
(
37
,
79
%
)
demonstrated
evidence
of
urologic
involvement
from
ECD
,
requiring
urologic
surgery
in
13
(
28
%
)
.
This
urologic
involvement
included
retroperitoneal
infiltration
(
28
,
60
%
)
,
worsening
lower
tract
urinary
symptoms
from
diabetes
insipidus
(
21
,
45
%
)
,
hydronephrosis
(
10
,
21
%
)
,
and
chronic
kidney
disease
(
18
,
38
%
)
.
The
incidence
of
urologic
involvement
with
ECD
is
higher
than
previously
reported
in
the
published
data
.
Urologists
should
be
aware
of
this
disorder
,
as
it
might
mimic
other
retroperitoneal
diseases
and
might
contribute
to
lower
urinary
tract
symptoms
,
hydronephrosis
,
renal
atrophy
,
and
chronic
kidney
disease
.
Diseases
Validation
Diseases presenting
"bone involvement"
symptom
cutaneous mastocytosis
dentin dysplasia
erdheim-chester disease
familial hypocalciuric hypercalcemia
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