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Blood pressure, fludrocortisone dose and plasma renin activity in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency followed from birth to 4Â years of age.
[congenital adrenal hyperplasia]
Infants
with
congenital
adrenal
hyperplasia
(
CAH
)
require
higher
doses
of
fludrocortisone
(
FC
)
due
to
physiological
mineralocorticoid
resistance
.
The
adequacy
of
mineralocorticoid
replacement
should
be
closely
monitored
to
avoid
hypertension
.
To
evaluate
blood
pressure
(
BP
)
in
infants
with
CAH
due
to
21
-
hydroxylase
deficiency
.
Thirty
-
three
patients
(
18
f
/
15
Â
m
)
diagnosed
by
newborn
screening
were
followed
until
the
age
of
4
Â
years
.
Mean
start
of
HC
and
FC
treatment
was
day
9
·
8
Â
±
Â
9
·
2
postnatally
.
Mean
daily
HC
dose
ranged
from
8
·
6
to
12
·
3
Â
mg
/
m
(
2
)
/
day
.
During
the
first
year
of
life
prevalence
of
systolic
hypertension
was
up
to
45
·
5
%
.
At
12
and
at
18
Â
months
,
BP
was
highest
.
Prevalence
of
systolic
hypertension
was
up
to
57
·
6
%
at
18
Â
months
of
age
.
After
24
Â
months
BP
levels
were
lower
and
at
48
Â
months
prevalence
of
hypertension
decreased
to
15
·
2
%
.
Systolic
and
diastolic
BP
correlated
significantly
with
the
administered
fludrocortisone
dose
(
r
Â
=
Â
0
·
3
,
P
Â
=
Â
0
·
005
)
,
but
not
with
body
mass
index
.
Hypertensive
children
received
significantly
higher
FC
doses
and
had
significantly
lower
plasma
renin
activity
during
the
study
period
.
High
prevalence
of
transient
,
most
likely
FC
induced
hypertension
was
found
in
young
children
with
classic
CAH
diagnosed
by
newborn
screening
.
The
changing
mineralocorticoid
sensitivity
in
infants
is
a
risk
factor
for
the
development
of
hypertension
in
patients
with
CAH
,
who
are
treated
with
FC
.
Therefore
suppressed
plasma
renin
activity
should
be
avoided
to
prevent
arterial
hypertension
.
Diseases
Validation
Diseases presenting
"physiological mineralocorticoid resistance"
symptom
congenital adrenal hyperplasia
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