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Relationship between final height and health outcomes in adults with congenital adrenal hyperplasia: United Kingdom congenital adrenal hyperplasia adult study executive (CaHASE).
[congenital adrenal hyperplasia]
Treatment
of
congenital
adrenal
hyperplasia
(
CAH
)
in
childhood
focuses
on
growth
and
development
and
adult
final
height
(
FH
)
is
a
measure
of
effective
treatment
.
We
hypothesized
that
shorter
adults
will
have
more
severe
underlying
disease
and
worse
health
outcomes
.
This
was
a
cross-sectional
analysis
of
199
adults
with
CAH
.
FH
and
quality
of
life
were
expressed
as
z-scores
adjusted
for
midparental
target
height
or
UK
population
height
.
FH
correlated
inversely
with
age
(
men
,
r
=
-
0
.
38
;
women
,
r
=
-
0
.
26
,
P
<
.
01
)
.
Men
and
women
had
z-scores
adjusted
for
midparental
target
height
of
-
2
and
-
1
,
respectively
,
and
both
groups
had
UK
population
height
z-scores
of
-
1
below
the
UK
population
(
P
<
.
01
)
.
In
women
,
FH
was
shorter
in
non-
salt-wasting
than
salt-wasting
classic
CAH
(
P
<
.
05
)
and
in
moderately
affected
genotype
group
B
women
than
either
more
severely
affected
groups
null
and
A
(
P
<
.
01
)
or
the
mildest
group
C
(
P
<
.
001
)
.
Short
stature
and
a
higher
prevalence
of
hypertension
were
observed
in
classic
CAH
patients
diagnosed
late
(
after
1
y
)
compared
with
those
diagnosed
early
and
in
women
treated
with
glucocorticoid
only
compared
with
those
treated
with
both
glucocorticoids
and
mineralocorticoids
(
P
<
.
05
)
.
FH
did
not
associate
with
insulin
sensitivity
,
lipid
profile
,
adiposity
,
or
quality
of
life
.
Adult
CAH
patients
remain
short
,
although
height
prognosis
has
improved
over
time
.
The
shortest
adults
are
those
diagnosed
late
with
moderate
severity
CAH
and
are
at
increased
risk
of
adult
hypertension
;
we
hypothesize
that
these
patients
are
exposed
in
childhood
to
high
androgens
and
/
or
excessive
glucocorticoids
with
potential
programming
of
hypertension
.
Another
possibility
is
inadequate
mineralocorticoid
treatment
early
in
life
in
the
late
-diagnosed
patient
group
.
Prospective
studies
are
now
required
to
examine
these
hypotheses
.
Diseases
Validation
Diseases presenting
"hypertension"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
aniridia
aromatase deficiency
cadasil
child syndrome
cohen syndrome
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cushing syndrome
cystinuria
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
fabry disease
familial hypocalciuric hypercalcemia
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
holt-oram syndrome
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
kallmann syndrome
kindler syndrome
lamellar ichthyosis
lymphangioleiomyomatosis
pendred syndrome
primary effusion lymphoma
scrub typhus
severe combined immunodeficiency
sneddon syndrome
typhoid
von hippel-lindau disease
well-differentiated liposarcoma
werner syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated