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[The clinical characteristics of 10 cases of adrenocorticotropic hormone- independent macronodular adrenal hyperplasia].
[cushing syndrome]
To
evaluate
the
clinical
characteristics
of
patients
with
adrenocorticotropin-independent
macronodular
adrenal
hyperplasia
(
AIMAH
)
.
A
total
of
10
AIMAH
cases
were
enrolled
in
this
retrospective
study
.
The
clinical
and
laboratory
findings
of
all
patients
were
collected
and
analyzed
.
All
patients
manifested
some
clinical
features
and
biochemical
evidence
of
Cushing
's
syndrome
.
The
plasma
adrenocorticotropic
hormone
(
ACTH
)
level
was
undetectable
in
all
the
patients
and
their
serum
cortisol
secretion
rhythm
was
abnormal
.
Low
and
high
-dose
dexamethasone
suppression
tests
failed
to
suppress
the
cortisol
secretion
.
The
bilateral
macronodular
adrenal
enlargement
was
shown
by
CT
/
magnetic
resonance
imaging
.
The
supine-upright
posture
test
was
positive
in
four
patients
.
Three
patients
were
performed
bilateral
adrenalectomy
,
five
were
unilateral
adrenalectomy
and
the
remaining
two
patients
were
taken
propranolol
.
All
the
patients
had
followed
up
for
10
to
89
months
.
Contralateral
adrenalectomy
was
performed
in
two
patients
with
recurrent
symptoms
after
unilateral
adrenalectomy
and
two
patients
given
propranolol
were
underwent
bilateral
adrenalectomy
when
their
symptoms
had
not
been
improved
or
recurred
.
AIMAH
is
a
relatively
rare
subtype
of
Cushing
's
syndrome
with
unique
clinical
and
laboratory
findings
.
Propranolol
is
a
good
choice
if
the
supine-upright
posture
test
is
positive
.
Unilateral
adrenalectomy
appears
to
be
an
effective
and
safe
alternative
treatment
for
AIMAH
.
Bilateral
adrenalectomy
could
be
performed
if
the
symptoms
have
not
been
improved
or
recurred
after
unilateral
adrenalectomy
.
Diseases
Validation
Diseases presenting
"unilateral adrenalectomy"
symptom
adrenal incidentaloma
cushing syndrome
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