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Differentiating between Cushing's disease and pseudo-Cushing's syndrome: comparison of four tests.
[cushing syndrome]
To
evaluate
the
diagnostic
performance
of
four
different
tests
in
order
to
differentiate
between
Cushing
's
disease
(
CD
)
and
pseudo-
Cushing
's
syndrome
(
PCS
)
.
In
this
prospective
study
,
a
total
of
73
patients
with
clinical
features
of
hypercortisolism
and
insufficient
suppression
of
serum
cortisol
after
1
 
mg
overnight
dexamethasone
and
/
or
an
elevated
excretion
of
cortisol
in
24
-
h
urine
samples
were
included
.
The
circadian
rhythm
of
serum
cortisol
levels
as
well
as
midnight
serum
cortisol
(
MserC
)
levels
were
assessed
in
all
73
patients
.
Late
-
night
salivary
cortisol
(
LNSC
)
concentrations
were
obtained
in
44
patients
.
The
dexamethasone-
CRH
(
Dex
-
CRH
)
test
was
performed
in
54
patients
.
FIFTY-THREE
PATIENTS
WERE
DIAGNOSED
WITH
CD
AND
SUBSEQUENTLY
TREATED
.
TWENTY
PATIENTS
WERE
CLASSIFIED
AS
HAVING
PSC
.
SERUM
CORTISOL
CIRCADIAN
RHYTHM
:
the
diurnal
rhythmicity
of
cortisol
secretion
was
retained
in
PCS
.
A
cortisol
midnight
:
morning
ratio
of
>
0
.
67
is
highly
suggestive
of
CD
(
positive
predictive
value
(
PPV
)
100
%
and
negative
predictive
value
(
NPV
)
73
%
)
.
MserC
concentration
>
243
 
nmol
/
l
has
a
PPV
of
98
%
in
predicting
true
CD
(
NPV
95
%
)
.
LNSC
level
>
9
.
3
 
nmol
/
l
predicted
CD
in
94
%
of
patients
(
NPV
100
%
)
.
Dex
-
CRH
test
:
after
2
days
of
dexamethasone
suppression
,
a
CRH
-stimulated
cortisol
level
>
87
 
nmol
/
l
(
T
=
15
 
min
)
resulted
in
a
PPV
of
100
%
and
an
NPV
of
90
%
.
The
Dex
-
CRH
test
as
well
as
a
single
measurement
of
cortisol
in
serum
or
saliva
at
late
(
mid-
)
night
demonstrated
high
diagnostic
accuracy
in
differentiating
PCS
from
true
CD
.
Diseases
Validation
Diseases presenting
"positive predictive value"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
carcinoma of the gallbladder
cholangiocarcinoma
congenital adrenal hyperplasia
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
esophageal adenocarcinoma
esophageal carcinoma
familial mediterranean fever
heparin-induced thrombocytopenia
hodgkin lymphoma, classical
phenylketonuria
thoracic outlet syndrome
typhoid
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