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Long-chain polyunsaturated fatty acids in plasma and erythrocyte membrane lipids of children with phenylketonuria after controlled linoleic acid intake.
[classical phenylketonuria]
It
has
been
reported
that
children
with
classical
phenylketonuria
(
PKU
)
have
reduced
levels
of
arachidonic
acid
(
AA
,
20
:
4
n-
6
)
and
docosahexaenoic
acid
(
DHA
,
22
:
6
n-
3
)
in
plasma
and
membrane
phospholipids
compared
to
controls
and
may
therefore
require
supplementation
.
However
,
it
is
not
established
that
these
changes
are
specific
for
PKU
.
They
may
as
well
be
attributed
to
the
specific
composition
of
a
largely
vegetarian
diet
used
for
dietary
PKU
treatment
.
We
therefore
investigated
the
fatty
acid
composition
of
plasma
phospholipids
(
PL
)
,
plasma
cholesterol
esters
(
CE
)
,
red
blood
cell
phosphatidylcholine
(
PC
)
,
and
red
blood
cell
phosphatidylethanolamine
(
PE
)
in
two
groups
of
PKU
patients
including
8
children
between
1
and
6
years
(
group
A
)
,
9
adolescents
between
11
and
18
years
(
group
B
)
,
and
20
age-matched
healthy
controls
.
Group
A
had
good
dietary
control
(
median
plasma
phenylalanine
272
mumol
/
L
during
the
last
6
months
before
phospholipid
analysis
)
while
median
phenylalanine
in
group
B
was
714
mumol
/
L
(
p
<
0
.
001
)
.
When
compared
to
age-matched
controls
,
group
A
showed
significantly
lower
DHA
levels
in
PE
(
4
.
21
vs
5
.
85
weight
%
(
wt
%
)
,
p
<
0
.
01
)
,
in
PC
(
1
.
02
vs
1
.
25
wt
%
,
p
<
0
.
05
)
and
in
CE
(
0
.
25
vs
0
.
54
wt
%
,
p
<
0
.
05
)
.
There
was
no
significant
difference
of
DHA
between
group
B
and
controls
.
AA
levels
were
similar
in
phospholipids
of
all
groups
.
We
conclude
that
reduced
levels
of
long
-chain
polyunsaturated
fatty
acids
in
PKU
patients
occur
only
in
those
patients
with
strict
dietary
therapy
with
respect
to
n-
3
fatty
acids
,
most
probably
caused
by
reduced
intake
of
n-
3
fatty
acids
.
Diseases
Validation
Diseases presenting
"largely vegetarian diet"
symptom
classical phenylketonuria
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