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Erdheim-Chester disease and pituitary involvement: a unique case and the literature.
[erdheim-chester disease]
An
early
thirties
man
diagnosed
with
Erdheim-
Chester
disease
(
ECD
)
was
simultaneously
disclosed
to
have
hypogonadotropic
hypogonadism
,
central
adrenal
insufficiency
,
and
GH
deficiency
in
addition
to
central
diabetes
insipidus
(
CDI
)
.
Pituitary
magnetic
resonance
imaging
(
MRI
)
showed
swelling
in
the
stalk
,
enlargement
of
the
anterior
lobe
with
delayed
enhancement
,
and
loss
of
high
intensity
of
the
posterior
lobe
on
T
1
-
weighted
images
,
suggesting
of
pituitary
involvement
of
ECD
.
Three
months
after
starting
treatment
with
interferon
α
and
zoledronic
acid
,
polyuria
and
polydipsia
were
ameliorated
without
DDAVP
,
accompanied
with
improvement
of
MRI
.
Simultaneously
technetium-
99
m
bone
scintigraphy
showed
improvement
,
accompanied
with
a
relief
of
bone
pain
and
high
fever
.
In
contrast
,
he
developed
secondary
hypothyroidism
with
slight
enlargement
of
anterior
pituitary
gland
without
relapse
of
CDI
,
suggesting
of
different
responses
to
treatment
with
interferon
α
between
anterior
pituitary
lobe
and
posterior
one
.
So
far
he
continues
to
be
replaced
with
deficient
hormone
replacement
therapy
.
As
for
bone
pain
,
it
remains
to
be
controlled
with
the
decreased
levels
of
bone
resorption
marker
with
decreased
abnormal
uptake
in
bone
scintigraphy
although
zoledronic
acid
was
discontinued
for
osteonecrosis
of
the
jaw
.
For
four
years
,
he
has
not
showed
new
involvement
at
other
organs
besides
bones
and
the
pituitary
.
While
CDI
is
known
to
be
very
common
in
ECD
,
improvement
of
CDI
has
been
reported
in
a
few
cases
.
Other
endocrine
manifestations
,
especially
with
detailed
endocrine
status
,
have
been
also
reported
in
limited
cases
.
Thus
we
report
this
case
and
review
the
literature
.
Diseases
Validation
Diseases presenting
"pituitary involvement"
symptom
erdheim-chester disease
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