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A randomized trial of long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria.
[classical phenylketonuria]
Forty
-
two
infants
(
20
males
,
22
females
)
with
classical
phenylketonuria
(
PKU
)
entered
a
prospective
,
double
-blind
,
randomized
study
to
investigate
the
effects
on
biochemical
and
physiological
outcomes
of
a
phenylalanine-free
infant
formula
containing
a
fat
blend
supplemented
with
the
long
-chain
polyunsaturated
fatty
acids
(
LC
-PUFA
)
,
docosahexaenoic
acid
(
DHA
,
C
2
2
:
6
n-
3
)
,
and
arachidonic
acid
(
AA
,
C
2
0
:
4
n-
6
)
.
Between
entry
and
20
weeks
(
entry
and
1
y
)
of
age
,
median
DHA
levels
in
erythrocyte
membrane
phospholipids
decreased
by
15
%
(
22
%
)
in
the
LC
-PUFA
supplemented
group
(
n
=
21
)
and
by
61
%
(
64
%
)
in
the
control
group
(
p
<
0
.
001
;
n
=
18
)
.
A
dietary
supply
of
LC
-PUFA
in
infants
with
PKU
prevents
the
decline
in
DHA
levels
associated
with
a
diet
supplying
minimal
sources
of
LC
-PUFA
.
DHA
status
in
turn
,
independent
of
diet
,
may
influence
the
maturation
of
the
visual
system
in
infants
with
PKU
.
Diseases
Validation
Diseases presenting
"a diet supplying minimal sources of lc-pufa"
symptom
classical phenylketonuria
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