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Bone mineral density, body composition and bone turnover in patients with congenital hypogonadotropic hypogonadism.
[kallmann syndrome]
Patients
with
congenital
hypogonadotropic
hypogonadism
(
HH
)
may
have
reduced
peak
bone
mass
in
early
adulthood
,
and
increased
risk
for
osteoporosis
despite
long
-term
hormonal
replacement
therapy
(
HRT
)
.
To
investigate
the
relationship
between
HRT
history
and
measures
of
bone
health
in
patients
with
HH
,
we
recruited
33
subjects
(
24
men
,
nine
women
;
mean
age
39
.
8
years
,
range
:
24
.
0
-
69
.
1
)
with
congenital
HH
(
Kallmann
syndrome
or
normosmic
HH
)
.
They
underwent
clinical
examination
,
were
interviewed
and
medical
charts
were
reviewed
.
Twenty
-
six
subjects
underwent
dual-energy
X-
ray
absorptiometry
for
evaluation
of
BMD
of
lumbar
spine
,
hip
,
femoral
neck
and
whole
body
;
body
composition
and
vertebral
morphology
were
evaluated
in
22
and
23
subjects
,
respectively
.
Circulating
PINP
,
ICTP
and
sex
hormone
levels
were
measured
.
HRT
history
clearly
associated
to
bone
health
:
BMDs
of
lumbar
spine
,
femoral
neck
,
hip
and
whole
body
were
lower
in
subjects
(
n
=
9
)
who
had
had
long
(
≥
5
years
)
treatment
pauses
or
low
dose
testosterone
(
T
)
treatment
as
compared
to
subjects
without
such
history
(
n
=
17
;
all
p
-values
<
0
.
05
)
.
In
addition
,
fat
mass
and
body
mass
index
(
BMI
)
were
significantly
higher
in
men
with
deficient
treatment
history
(
median
fat
mass
:
37
.
5
vs
.
23
.
1
%
,
p
=
0
.
005
;
BMI
:
32
.
6
vs
.
25
.
2
kg
/
m
(
2
)
,
p
<
0
.
05
)
.
Serum
PINP
correlated
with
ICTP
(
r
(
s
)
=
0
.
61
;
p
<
0
.
005
)
in
men
,
but
these
markers
correlated
neither
with
circulating
T
,
nor
with
serum
estradiol
levels
in
women
.
In
conclusion
,
patients
with
congenital
HH
require
life-
long
follow-up
to
avoid
inadequate
HRT
,
long
treatment
pauses
and
further
morbidity
.
Diseases
Validation
Diseases presenting
"long treatment"
symptom
fabry disease
kallmann syndrome
krabbe disease
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