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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
"cardiovascular disease"
symptom
aromatase deficiency
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cushing syndrome
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
heparin-induced thrombocytopenia
hodgkin lymphoma, classical
inclusion body myositis
phenylketonuria
werner syndrome
This symptom has already been validated