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Hypogonadotropic hypogonadism revisited.
[kallmann syndrome]
Impaired
testicular
function
,
i
.
e
.
,
hypogonadism
,
can
result
from
a
primary
testicular
disorder
(
hypergonadotropic
)
or
occur
secondary
to
hypothalamic
-
pituitary
dysfunction
(
hypogonadotropic
)
.
Hypogonadotropic
hypogonadism
can
be
congenital
or
acquired
.
Congenital
hypogonadotropic
hypogonadism
is
divided
into
anosmic
hypogonadotropic
hypogonadism
(
Kallmann
syndrome
)
and
congenital
normosmic
isolated
hypogonadotropic
hypogonadism
(
idiopathic
hypogonadotropic
hypogonadism
)
.
The
incidence
of
congenital
hypogonadotropic
hypogonadism
is
approximately
1
-
10
:
100
,
000
live
births
,
and
approximately
2
/
3
and
1
/
3
of
cases
are
caused
by
Kallmann
syndrome
(
KS
)
and
idiopathic
hypogonadotropic
hypogonadism
,
respectively
.
Acquired
hypogonadotropic
hypogonadism
can
be
caused
by
drugs
,
infiltrative
or
infectious
pituitary
lesions
,
hyperprolactinemia
,
encephalic
trauma
,
pituitary
/
brain
radiation
,
exhausting
exercise
,
abusive
alcohol
or
illicit
drug
intake
,
and
systemic
diseases
such
as
hemochromatosis
,
sarcoidosis
and
histiocytosis
X
.
The
clinical
characteristics
of
hypogonadotropic
hypogonadism
are
androgen
deficiency
and
a
lack
/
delay
/
stop
of
pubertal
sexual
maturation
.
Low
blood
testosterone
levels
and
low
pituitary
hormone
levels
confirm
the
hypogonadotropic
hypogonadism
diagnosis
.
A
prolonged
stimulated
intravenous
GnRH
test
can
be
useful
.
In
Kallmann
syndrome
,
cerebral
MRI
can
show
an
anomalous
morphology
or
even
absence
of
the
olfactory
bulb
.
Therapy
for
hypogonadotropic
hypogonadism
depends
on
the
patient
's
desire
for
future
fertility
.
Hormone
replacement
with
testosterone
is
the
classic
treatment
for
hypogonadism
.
Androgen
replacement
is
indicated
for
men
who
already
have
children
or
have
no
desire
to
induce
pregnancy
,
and
testosterone
therapy
is
used
to
reverse
the
symptoms
and
signs
of
hypogonadism
.
Conversely
,
GnRH
or
gonadotropin
therapies
are
the
best
options
for
men
wishing
to
have
children
.
Hypogonadotropic
hypogonadism
is
one
of
the
rare
conditions
in
which
specific
medical
treatment
can
reverse
infertility
.
When
an
unassisted
pregnancy
is
not
achieved
,
assisted
reproductive
techniques
ranging
from
intrauterine
insemination
to
in
vitro
fertilization
to
the
acquisition
of
viable
sperm
from
the
ejaculate
or
directly
from
the
testes
through
testicular
sperm
extraction
or
testicular
microdissection
can
also
be
used
,
depending
on
the
woman
's
potential
for
pregnancy
and
the
quality
and
quantity
of
the
sperm
.
Diseases
Validation
Diseases presenting
"low blood testosterone levels"
symptom
kallmann syndrome
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