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Computed tomography of the anterior skull base in Kallmann syndrome reveals specific ethmoid bone abnormalities associated with olfactory bulb defects.
[kallmann syndrome]
Kallmann
syndrome
(
KS
)
is
characterized
by
congenital
hypogonadotropic
hypogonadism
(
CHH
)
and
an
impaired
sense
of
smell
related
to
defective
development
of
the
olfactory
system
.
The
aim
of
the
study
was
to
use
high
-resolution
computed
tomography
(
CT
)
to
detect
specific
abnormalities
in
the
ethmoid
bone
region
surrounding
the
olfactory
bulbs
in
patients
with
KS
.
Thirty
-
seven
KS
patients
were
compared
to
normosmic
CHH
(
nCHH
)
patients
(
n
=
15
)
and
controls
(
n
=
30
)
of
similar
age
.
We
conducted
a
prospective
study
in
a
single
referral
center
.
Subjects
underwent
CT
in
bone
windows
with
axial
,
coronal
,
and
sagittal
reconstructions
centered
on
the
olfactory
fossa
(
OF
)
and
cribriform
plate
(
CP
)
.
We
characterized
the
OF
structure
by
measuring
OF
height
,
width
,
and
surface
area
and
a
series
of
angles
.
The
CP
foramina
were
counted
bilaterally
.
Olfactory
bulb
magnetic
resonance
imaging
,
performed
in
parallel
,
was
compared
with
CT
findings
.
OF
height
,
width
,
and
surface
area
were
all
significantly
lower
in
KS
patients
than
in
nCHH
patients
and
controls
(
P
<
.
0001
)
.
KS
patients
also
had
wider
angles
than
nCHH
patients
and
controls
(
P
<
.
0001
)
.
KS
subjects
with
olfactory
bulb
agenesis
on
magnetic
resonance
imaging
or
who
harbored
KAL
1
mutations
had
the
most
marked
changes
in
OF
measurements
and
angles
.
Coronal
OF
height
distinguished
KS
patients
from
controls
with
the
best
sensitivity
and
specificity
.
The
mean
number
of
CP
foramina
was
similar
in
KS
,
nCHH
,
and
control
subjects
.
KS
is
associated
with
specific
ethmoid
bone
abnormalities
.
The
preserved
number
of
CP
foramina
in
KS
patients
suggests
that
the
integrity
of
olfactory
structures
is
not
mandatory
for
their
formation
during
fetal
development
or
their
maintenance
in
adult
life
.
Diseases
Validation
Diseases presenting
"bone abnormalities"
symptom
alpha-thalassemia
holt-oram syndrome
kallmann syndrome
pendred syndrome
proteus syndrome
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