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Tall stature without growth hormone: four male patients with aromatase deficiency.
[aromatase deficiency]
From
preliminary
observations
,
GH-IGF-
I
seems
to
be
compromised
in
men
with
aromatase
deficiency
.
The
GH
deficiency
(
GHD
)
coexists
paradoxically
with
tall
stature
,
raising
the
question
whether
or
not
a
true
GHD
is
part
of
this
rare
syndrome
.
To
evaluate
the
GH
secretion
in
aromatase-
deficient
men
,
their
GH
response
to
the
GHRH
plus
arginine
(
GHRH
-ARG
)
test
was
compared
with
that
of
normal
subjects
.
The
effect
of
estrogen
replacement
treatment
on
the
GH-IGF-
I
axis
in
aromatase-
deficient
men
was
evaluated
before
and
during
therapy
.
A
case-control
study
was
conducted
.
Four
adult
men
with
aromatase
deficiency
were
compared
with
12
normal
subjects
.
We
measured
the
GH
response
to
GHRH
-ARG
in
aromatase-
deficient
men
(
at
baseline
and
during
estrogen
treatment
)
and
in
normal
subjects
.
Basal
serum
IGF-
I
was
measured
in
both
patients
and
controls
.
The
response
of
GH
to
GHRH
-ARG
was
severely
impaired
in
men
with
aromatase
deficiency
and
resulted
in
significantly
lower
(
P
<
0
.
001
)
levels
than
in
normal
subjects
.
Although
normal
,
serum
IGF-
I
levels
were
also
significantly
lower
(
P
<
0
.
001
)
than
in
normal
subjects
.
Both
GH
peak
and
IGF-
I
concentrations
were
not
modified
by
estrogen
therapy
in
men
with
aromatase
deficiency
.
In
aromatase-
deficient
men
,
GH
response
to
potent
provocative
stimuli
is
impaired
and
is
not
restored
by
exogenous
estrogens
.
Furthermore
,
a
tall
stature
may
be
reached
,
notwithstanding
the
coexistence
of
GHD
,
if
a
prolonged
time
for
growth
is
available
due
to
a
delay
in
bone
maturation
,
and
other
growth
factors
different
from
GH
(
mainly
insulin
)
promote
growth
.
Diseases
Validation
Diseases presenting
"gh response to potent provocative stimuli is impaired and is not restored by exogenous estrogens"
symptom
aromatase deficiency
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