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Free asymmetric dimethylarginine (ADMA) is low in children and adolescents with classical phenylketonuria (PKU).
[classical phenylketonuria]
Free
asymmetric
dimethylarginine
(
ADMA
)
is
a
competitive
inhibitor
of
the
nitric
oxide
synthases
(
NOS
)
.
Suppression
of
nitric
oxide
(
NO
)
synthesis
increases
the
risk
of
atherosclerosis
.
Nevertheless
,
in
the
condition
of
oxidative
stress
,
NOS
blockade
by
ADMA
may
exert
protective
effects
.
Protein
metabolism
is
altered
in
patients
with
phenylketonuria
(
PKU
)
on
dietary
treatment
and
as
shown
recently
,
oxidative
stress
is
high
in
PKU
.
Since
free
ADMA
concentrations
are
determined
by
both
protein
metabolism
and
oxidative
stress
we
hypothesized
,
that
free
ADMA
levels
may
be
elevated
in
PKU
patients
.
Sixteen
patientswith
PKU
on
dietary
treatment
(
mean
age
10
.
1
±
5
.
2
yrs
)
,
and
91
healthy
children
(
mean
age
11
.
6
±
3
.
7
yrs
)
participated
in
a
cross
sectional
study
.
ADMA
,
total
homocysteine
(
tHcy
)
and
blood
glucose
were
lower
and
the
L-
arginine
/
ADMA
ratio
was
higher
in
PKU
patients
compared
to
controls
.
No
significant
correlation
was
present
between
phenylalanine
(
Phe
)
concentrations
,
protein
intake
,
and
lipid
profile
,
history
of
cardiovascular
disease
or
ADMA
.
In
contrast
to
our
hypothesis
,
ADMAwas
lower
and
the
L-
arginine
/
ADMA
ratio
was
higher
in
PKU
patients
.
Therefore
,
in
PKU
patients
,
the
regulating
function
of
ADMA
on
NO
synthesis
is
altered
and
may
thus
contribute
to
oxidative
stress
.
Diseases
Validation
Diseases presenting
"atherosclerosis"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
alexander disease
aromatase deficiency
benign recurrent intrahepatic cholestasis
cadasil
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cushing syndrome
erdheim-chester disease
familial mediterranean fever
harlequin ichthyosis
hereditary cerebral hemorrhage with amyloidosis
inclusion body myositis
sneddon syndrome
werner syndrome
zellweger syndrome
This symptom has already been validated