Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Impact and timing of bilateral adrenalectomy for refractory adrenocorticotropic hormone-dependent Cushing’s syndrome.
[cushing syndrome]
In
patients
with
refractory
adrenocorticotropic
hormone-dependent
Cushing
’
s
syndrome
,
we
evaluated
steroidogenesis
inhibition
(
SI
)
and
bilateral
adrenalectomy
(
BA
)
to
predict
which
patients
might
benefit
most
from
each
treatment
modality
.
Clinical
data
from
patients
treated
1970
-
2012
were
reviewed
retrospectively
by
treatment
group
(
SI
or
SI
+
BA
)
.
Validated
severity
scales
were
used
to
calculate
metabolic
(
M
)
score
(
hypokalemia
,
hyperglycemia
,
hypertension
,
proximal
muscle
weakness
)
and
adverse
events
(
AE
)
score
(
thrombosis
,
fracture
,
infection
)
.
A
total
of
65
patients
(
16
pituitary
,
49
ectopic
)
were
treated
with
SI
+
BA
(
n
=
21
,
32
%
)
or
SI
alone
(
n
=
44
,
68
%
)
.
Presenting
M
scores
and
source
of
adrenocorticotropic
hormone
excess
(
ectopic
versus
pituitary
)
were
similar
.
Both
groups
improved
metabolically
after
treatment
.
Over
one
-
third
of
AEs
in
the
SI
+
BA
group
occurred
within
12
months
of
presentation
.
Half
(
n
=
24
,
55
%
)
of
the
patients
treated
with
SI
died
(
median
survival
,
24
.
0
months
)
.
Steroid
excess
contributed
to
71
%
of
complications
.
Six
SI
+
BA
patients
died
(
29
%
)
,
including
all
3
patients
with
recurrent
Cushing
’
s
syndrome
after
BA
.
Minor
perioperative
complications
occurred
in
7
patients
(
33
%
)
.
Posttreatment
M
and
AE
scores
improved
for
all
patients
and
70
%
of
AEs
occurred
in
SI
+
BA
patients
within
12
months
of
presentation
,
emphasizing
the
importance
of
early
operative
intervention
.
These
data
argue
for
the
safety
and
efficacy
of
early
BA
in
selected
patients
with
uncontrollable
Cushing
’
s
syndrome
.
Diseases
Validation
Diseases presenting
"hypertension"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
aniridia
aromatase deficiency
cadasil
child syndrome
cohen syndrome
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cushing syndrome
cystinuria
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
fabry disease
familial hypocalciuric hypercalcemia
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
holt-oram syndrome
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
kallmann syndrome
kindler syndrome
lamellar ichthyosis
lymphangioleiomyomatosis
pendred syndrome
primary effusion lymphoma
scrub typhus
severe combined immunodeficiency
sneddon syndrome
typhoid
von hippel-lindau disease
well-differentiated liposarcoma
werner syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated