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Sex steroids, precursors and metabolites deficiencies in men with isolated hypogonadotropic hypogonadism and panhypopituitarism: a GCMS-based comparative study.
[kallmann syndrome]
Context
:
Both
testicular
and
adrenal
steroid
secretions
are
impaired
in
men
with
panhypopituitarism
(
Hypo-
Pit
)
,
whereas
only
testicular
steroid
secretion
is
impaired
in
men
with
isolated
gonadotropin
deficiency
(
IHH
)
caused
by
normosmic
congenital
hypogonadotropic
hypogonadism
(
nCHH
)
or
Kallmann
syndrome
(
KS
)
.
Objective
:
To
compare
serum
levels
of
sex
steroids
,
precursors
and
metabolites
between
men
with
complete
IHH
and
those
with
Hypo-
Pit
.
Patients
:
We
studied
42
healthy
men
,
16
untreated
men
with
IHH
(
nCHH
/
KS
)
and
23
men
with
Hypo-
Pit
(
14
with
craniopharyngioma
,
9
with
congenital
hypopituitarism
)
receiving
hydrocortisone
,
thyroxin
and
GH
replacement
therapy
but
not
testosterone
.
Methods
:
Gas
chromatography
/
mass
spectrometry
(
GCMS
)
was
used
to
measure
serum
levels
of
sex
steroids
(
testosterone
(
T
)
,
dihydrotestosterone
(
DHT
)
,
and
estradiol
(
E
2
)
)
,
their
precursors
(
pregnenolone
,
17
hydroxy-pregnenolone
,
dehydroepiandrosterone
,
androstenediol
,
progesterone
,
17
hydroxy-progesterone
,
androstenedione
)
and
their
metabolites
(
androsterone
,
estrone
,
estrone
sulfate
)
,
as
well
as
pregnenolone
and
DHEA
sulfate
esters
(
PREGS
and
DHEAS
)
.
Results
:
All
the
above-mentioned
steroids
,
and
notably
T
,
DHT
and
E
2
,
were
significantly
lower
in
IHH
patients
than
in
controls
but
remained
well
above
the
detection
limit
of
the
relevant
assays
.
In
Hypo-
Pit
men
,
all
these
steroids
were
dramatically
and
significantly
lower
than
in
IHH
.
Interestingly
,
T
,
DHT
and
E
2
,
as
well
as
PREGS
and
DHEAS
,
were
undetectable
or
barely
detectable
in
Hypo-
Pit
men
.
Conclusions
:
Steroid
deficiencies
are
marked
but
partial
in
men
with
complete
IHH
.
In
contrast
,
men
with
Hypo-
Pit
have
a
very
severe
overall
steroid
deficiency
.
These
deficiencies
could
affect
health
and
quality
of
life
.
Diseases
Validation
Diseases presenting
"adrenal steroid secretions"
symptom
kallmann syndrome
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