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A Retrospective Analysis of the Growth Pattern in Patients with Salt-wasting 21-Hydroxylase Deficiency.
[congenital adrenal hyperplasia]
The
objective
of
this
study
was
to
investigate
the
growth
pattern
of
children
with
the
salt-wasting
form
of
congenital
adrenal
hyperplasia
caused
by
21
-
hydroxylase
deficiency
(
21
-
OHD
)
.
We
reviewed
the
medical
records
of
13
patients
in
whom
salt-wasting
21
-
OHD
was
diagnosed
during
the
first
2
mo
of
life
at
our
hospital
from
1980
through
2008
.
Six
reached
adult
height
.
Growth
patterns
,
bone
age
,
biochemical
data
,
and
the
hydrocortisone
dose
at
each
growth
stage
were
analyzed
retrospectively
.
The
mean
adult
height
was
155
.
1
±
6
.
5
cm
(
mean
±
SD
)
in
females
and
158
.
1
±
7
.
1
cm
in
males
.
Although
length
at
birth
was
normal
or
longer
than
the
national
mean
in
almost
all
patients
,
the
mean
height
SD
score
of
both
boys
and
girls
decreased
to
below
0
SD
during
infancy
.
Subsequently
,
both
boys
and
girls
transiently
showed
growth
acceleration
and
reached
their
peak
growth
velocity
at
3
-
10
yr
of
age
.
In
conclusion
,
in
addition
to
suppression
of
growth
during
infancy
,
there
was
inappropriate
growth
acceleration
during
childhood
.
Especially
from
3
mo
to
3
yr
of
age
,
decreasing
the
hydrocortisone
dose
in
patients
who
exhibit
slower
growth
may
lead
to
satisfactory
height
outcomes
.
Also
,
strict
adjustment
of
the
hydrocortisone
dose
to
avoid
accelerated
growth
from
childhood
to
adolescence
might
improve
adult
height
outcomes
of
patients
with
21
-
OHD
.
Diseases
Validation
Diseases presenting
"bone age"
symptom
achondroplasia
aromatase deficiency
congenital adrenal hyperplasia
cushing syndrome
kabuki syndrome
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