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Performance of phalangeal quantitative ultrasound parameters in the evaluation of reduced bone mineral density assessed by DX in patients with 21 hydroxylase deficiency.
[congenital adrenal hyperplasia]
The
purpose
of
this
study
was
to
verify
the
performance
of
quantitative
ultrasound
(
QUS
)
parameters
of
proximal
phalanges
in
the
evaluation
of
reduced
bone
mineral
density
(
BMD
)
in
patients
with
congenital
adrenal
hyperplasia
due
to
21
-
hydroxylase
deficiency
(
21
OHD
)
.
Seventy
patients
with
21
OHD
(
41
females
and
29
males
)
,
aged
between
6
-
27
y
were
assessed
.
The
QUS
measurements
,
amplitude-dependent
speed
of
sound
(
AD
-SoS
)
,
bone
transmission
time
(
BTT
)
,
and
ultrasound
bone
profile
index
(
UBPI
)
were
obtained
using
the
BMD
Sonic
device
(
IGEA
,
Carpi
,
Italy
)
on
the
last
four
proximal
phalanges
in
the
non-dominant
hand
.
BMD
was
determined
by
dual
energy
X-
ray
(
DXA
)
across
the
total
body
and
lumbar
spine
(
LS
)
.
Total
body
and
LS
BMD
were
positively
correlated
to
UBPI
,
BTT
and
AD
-SoS
(
correlation
coefficients
ranged
from
0
.
59
-
0
.
72
,
p
<
0
.
001
)
.
In
contrast
,
when
comparing
patients
with
normal
and
low
(
Z-
score
<
-
2
)
BMD
,
no
differences
were
found
in
the
QUS
parameters
.
Furthermore
,
UBPI
,
BTT
and
AD
-SoS
measurements
were
not
effective
for
diagnosing
patients
with
reduced
BMD
by
receiver
operator
characteristic
curve
parameters
.
Although
the
AD
-SoS
,
BTT
and
UBPI
showed
significant
correlations
with
the
data
obtained
by
DXA
,
they
were
not
effective
for
diagnosing
reduced
bone
mass
in
patients
with
21
OHD
.
Diseases
Validation
Diseases presenting
"bone mineral density"
symptom
achondroplasia
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
aromatase deficiency
congenital adrenal hyperplasia
cushing syndrome
dentinogenesis imperfecta
erythropoietic protoporphyria
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
kallmann syndrome
lamellar ichthyosis
phenylketonuria
primary hyperoxaluria type 1
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