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Hypothalamic-pituitary-gonadal axis in two men with aromatase deficiency: evidence that circulating estrogens are required at the hypothalamic level for the integrity of gonadotropin negative feedback.
[aromatase deficiency]
In
men
,
the
feedback
of
gonadotropins
is
regulated
by
estrogens
that
come
from
the
aromatization
of
testosterone
,
but
the
relative
contribution
to
the
inhibition
of
LH
and
FSH
secretion
by
the
amount
of
locally
produced
estrogens
within
the
hypothalamus
and
/
or
the
pituitary
,
and
the
amount
of
circulating
estrogens
still
remains
unknown
.
In
order
to
evaluate
the
effect
of
regulation
induced
by
estradiol
on
the
hypothalamic
-
pituitary
-
gonadal
(
HPG
)
axis
,
we
studied
the
pulsatility
of
LH
and
FSH
in
two
aromatase-
deficient
men
(
called
subject
1
and
subject
2
)
,
in
which
the
production
rate
of
estrogen
(
both
local
and
circulating
)
is
completely
,
or
at
least
severely
,
impaired
.
FSH
and
LH
were
evaluated
in
terms
of
their
pulsated
secretion
and
as
GnRH-stimulated
secretion
in
two
phases
:
phase
1
,
before
estrogen
treatment
;
and
phase
2
,
during
estrogen
treatment
with
25
microg
transdermal
estradiol
twice
weekly
.
Blood
samples
were
taken
during
phase
1
and
phase
2
at
0800
h
for
basal
measurements
of
LH
,
FSH
,
inhibin
B
,
testosterone
,
and
estradiol
.
The
analysis
of
the
pulsatility
of
LH
and
FSH
was
performed
by
sampling
every
10
min
for
8
h
in
the
two
phases
.
Gonadotropin
response
to
GnRH-stimulation
test
was
studied
by
serial
standard
sampling
after
100
microg
GnRH
i
.
v
.
bolus
in
phases
1
and
2
.
Estrogen
treatment
led
to
a
significant
reduction
in
both
LH-pulsated
frequency
(
7
.
5
+
/
-
0
.
7
in
phase
1
,
4
.
5
+
/
-
0
.
7
in
phase
2
)
and
amplitudes
(
3
.
5
+
/
-
0
.
006
in
phase
1
,
1
.
9
+
/
-
0
.
4
in
phase
2
)
of
peaks
,
whereas
FSH
showed
only
a
conspicuous
reduction
in
serum
levels
and
a
trend
towards
the
reduction
of
the
amplitudes
of
its
peaks
without
modification
of
the
frequency
of
the
pulses
.
Both
testosterone
and
gonadotropins
decreased
during
phase
2
,
whereas
estradiol
reached
the
normal
range
in
both
subjects
.
Transdermal
estradiol
treatment
significantly
lowered
the
peaks
of
both
serum
LH
and
FSH
after
GnRH
as
well
as
the
incremental
area
under
the
curve
after
GnRH
administration
in
both
subjects
.
Basal
serum
inhibin
B
levels
were
slightly
higher
before
transdermal
estradiol
treatment
(
phase
1
)
than
during
estrogen
treatment
(
phase
2
)
in
both
subjects
.
The
administration
of
estrogen
to
aromatase-
deficient
men
discloses
the
effects
of
circulating
estrogens
on
LH
secretion
,
exerted
both
at
pituitary
level
,
as
shown
by
the
decrease
of
basal
and
GnRH-stimulated
secretion
of
LH
and
the
LH
pulsed
amplitude
,
and
at
hypothalamic
level
as
shown
by
the
reduction
of
the
frequency
of
LH
pulses
.
The
present
study
,
coupling
the
outcomes
of
basal
,
GnRH-stimulated
and
the
pulsatile
evaluation
of
LH
and
FSH
secretion
in
two
aromatase-
deficient
men
,
demonstrates
that
circulating
estrogens
play
an
inhibitory
role
in
LH
secretion
by
acting
on
the
hypothalamus
and
the
pituitary
gland
of
men
.
The
discrepancy
among
testosterone
levels
,
the
arrest
of
spermatogenesis
and
a
slightly
inappropriate
respective
increase
of
serum
FSH
(
lower
than
expected
)
suggests
a
possible
role
of
estrogens
in
the
priming
and
the
maturation
of
HPG
axis
in
men
,
an
event
that
has
never
occurred
in
these
two
subjects
as
a
consequence
of
chronic
estrogen
deprivation
.
Diseases
Validation
Diseases presenting
"as shown by the decrease of basal and gnrh-stimulated secretion of lh and the lh pulsed amplitude"
symptom
aromatase deficiency
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