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Congenital hypogonadotropic hypogonadism and Kallmann syndrome as models for studying hormonal regulation of human testicular endocrine functions.
[kallmann syndrome]
Men
with
Kallmann
syndrome
(
KS
)
and
those
with
congenital
isolated
hypogonadotropic
hypogonadism
with
normal
olfaction
share
a
chronic
,
usually
profound
deficit
,
in
FSH
and
LH
,
the
two
pituitary
gonadotropins
.
Many
studies
indicate
that
this
gonadotropin
deficiency
is
already
present
during
fetal
life
,
thus
explaining
the
micropenis
,
cryptorchidism
and
marked
testicular
hypotrophy
already
present
at
birth
.
In
addition
,
neonatal
activation
of
gonadotropin
secretion
is
compromised
in
boys
with
severe
CHH
/
Kallmann
,
preventing
the
first
phase
of
postnatal
testicular
activation
.
Finally
,
CHH
is
characterized
by
the
persistence
,
in
the
vast
majority
of
cases
,
of
gonadotropin
deficiency
at
the
time
of
puberty
and
during
adulthood
.
This
prevents
the
normal
pubertal
testicular
reactivation
required
for
physiological
sex
steroid
and
testicular
peptide
production
,
and
for
spermatogenesis
.
CHH
/
KS
thus
represents
a
pathological
paradigm
that
can
help
to
unravel
,
in
vivo
,
the
role
of
each
gonadotropin
in
human
testicular
exocrine
and
endocrine
functions
at
different
stages
of
development
.
Recombinant
gonadotropins
with
pure
LH
or
FSH
activity
have
been
used
to
stimulate
Leydig
's
cells
and
Sertoli
's
cells
,
respectively
,
and
thereby
to
clarify
their
paracrine
interaction
in
vivo
.
The
effects
of
these
pharmacological
probes
can
be
assessed
by
measuring
the
changes
they
provoke
in
circulating
testicular
hormone
concentrations
.
This
review
discusses
the
impact
of
chronic
gonadotropin
deficiency
on
the
endocrine
functions
of
the
interstitial
compartment
,
which
contains
testosterone-
,
estradiol-
and
INSL
3
-
secreting
Leydig
's
cells
.
It
also
examines
the
regulation
of
inhibin
B
and
anti-
Mullerian
hormone
(
AMH
)
secretion
in
the
seminiferous
tubules
,
and
the
insights
provided
by
studies
of
human
testicular
stimulation
with
recombinant
gonadotropins
,
used
either
individually
or
in
combination
.
Diseases
Validation
Diseases presenting
"testicular peptide production"
symptom
kallmann syndrome
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