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Salivary morning androstenedione and 17α-OH progesterone levels in childhood and puberty in patients with classic congenital adrenal hyperplasia.
[congenital adrenal hyperplasia]
Abstract
Background
:
Treatment
of
congenital
adrenal
hyperplasia
due
to
21
-
hydroxylase
deficiency
can
be
monitored
by
salivary
androstenedione
(
A-
dione
)
and
17
α-hydroxyprogesterone
(
17
OHP
)
levels
.
There
are
no
objective
criteria
for
setting
relevant
target
values
or
data
on
changes
of
17
OHP
and
A-
dione
during
monitoring
.
Methods
:
We
evaluated
A-
dione
and
17
OHP
levels
in
nearly
2000
salivary
samples
collected
during
long
-term
treatment
of
84
paediatric
patients
with
classic
21
-
hydroxylase
deficiency
.
Results
:
A-
dione
and
17
OHP
levels
and
its
ratio
17
OHP
/
A-
dione
remained
constant
from
4
to
11
years
with
no
sex-related
differences
.
During
puberty
,
A-
dione
and
17
OHP
levels
both
increased
,
starting
at
earlier
age
in
girls
than
in
boys
.
The
ratio
17
OHP
/
A-
dione
declined
.
Normalised
A-
dione
concomitant
with
elevated
17
OHP
[
1
.
43
nmol
/
L
(
0
.
46
-
4
.
41
)
during
prepuberty
;
2
.
36
nmol
/
L
(
0
.
63
-
8
.
89
)
for
boys
and
1
.
99
nmol
/
L
(
0
.
32
-
6
.
98
)
for
girls
during
puberty
]
could
be
obtained
with
overall
median
glucocorticoid
doses
of
11
-
15
mg
/
m
2
/
day
.
A-
dione
levels
above
the
upper
reference
limit
(
URL
)
,
suggesting
undertreatment
,
coincided
with
17
OHP
levels
≥
10
times
URL
.
The
percentage
of
A-
dione
levels
above
URL
was
16
%
at
ages
4
-
8
years
,
but
increased
to
31
%
for
girls
at
16
years
and
46
%
for
boys
at
17
years
.
Conclusions
:
Normalised
A-
dione
consistent
with
17
OHP
three
times
URL
during
prepuberty
and
normalised
A-
dione
consistent
with
4
-
6
times
URL
during
puberty
could
be
obtained
by
moderate
glucocorticoid
dosages
.
A
constant
17
OHP
/
A-
dione
ratio
during
prepuberty
suggested
absence
of
adrenarche
.
During
puberty
,
a
higher
percentage
of
samples
met
the
criteria
for
undertreatment
,
especially
of
boys
.
Diseases
Validation
Diseases presenting
"long-term treatment"
symptom
classical phenylketonuria
congenital adrenal hyperplasia
dystrophic epidermolysis bullosa
fabry disease
homocystinuria without methylmalonic aciduria
phenylketonuria
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