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An objective olfactory evaluation and its correlation with magnetic resonance imaging findings in Asian Indian patients with idiopathic hypogonadotropic hypogonadism.
[kallmann syndrome]
Idiopathic
hypogonadotropic
hypogonadism
(
IHH
)
can
be
associated
with
subnormal
sense
of
smell
.
The
objective
of
our
study
was
to
determine
if
there
is
a
correlation
between
the
olfactory
phenotype
(
clinical
smell
test
)
of
IHH
patients
and
structural
abnormalities
in
the
olfactory
apparatus
on
magnetic
resonance
imaging
(
MRI
)
.
This
was
a
single
-center
prospective
case
control
study
.
Forty
-
one
IHH
patients
underwent
a
brief
University
of
Pennsylvania
Smell
Identification
Test
(
UPSIT
)
and
an
MRI
of
the
olfactory
apparatus
.
The
size
of
the
olfactory
sulcus
and
bulb
were
quantified
and
compared
with
the
normative
data
of
40
controls
.
Agreement
between
UPSIT
and
MRI
results
was
assessed
using
the
kappa
index
.
MRI
showed
that
the
olfactory
apparatus
was
normal
in
17
patients
,
hypoplastic
in
14
,
and
aplastic
in
10
.
All
13
patients
who
complained
of
anosmia
and
12
of
28
patients
who
reported
normosmia
had
a
low
UPSIT
score
.
Thus
,
25
patients
had
Kallmann
syndrome
(
KS
)
and
16
were
normosmic
IHH
(
nIHH
)
.
MRI
revealed
abnormalities
in
68
%
of
KS
and
37
.
5
%
of
nIHH
patients
.
The
MRI
abnormalities
in
KS
patients
were
aplasia
(
56
%
)
and
hypoplasia
(
44
%
)
.
All
6
nIHH
patients
with
abnormal
MRI
had
hypoplasia
.
A
significant
positive
correlation
(
r
=
0
.
61
;
P
<
.
01
)
between
olfactory
bulb
volume
(
from
MRI
)
and
smell-test
score
was
found
,
and
there
was
good
agreement
(
kappa
index
,
0
.
72
)
between
anosmia
and
the
presence
of
an
aplastic
olfactory
apparatus
.
Self-reporting
of
the
sense
of
smell
significantly
underestimates
olfactory
phenotype
;
hence
,
we
recommend
an
objective
smell
test
to
distinguish
KS
from
nIHH
.
Olfactory
phenotype
correlates
well
with
MRI
quantification
of
the
olfactory
apparatus
in
IHH
.
Diseases
Validation
Diseases presenting
"significant positive correlation"
symptom
acute rheumatic fever
congenital adrenal hyperplasia
familial mediterranean fever
kallmann syndrome
trochlear dysplasia
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