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Plasma micronutrient concentrations in infants undergoing therapy for phenylketonuria.
[classical phenylketonuria]
Twenty
-
seven
infants
with
classical
phenylketonuria
were
evaluated
longitudinally
for
6
mo
while
ingesting
Phenex-
1
Amino
Acid
Modified
Medical
Food
With
Iron
as
their
primary
protein
source
.
Intake
of
selected
nutrients
and
biochemical
indices
of
trace
and
ultratrace
mineral
status
and
plasma
retinol
and
alpha-tocopherol
concentrations
were
evaluated
.
The
means
of
iron
status
indices
(
complete
blood
count
,
plasma
ferritin
,
iron
,
transferrin
saturation
,
total
iron
binding
capacity
)
and
the
plasma
concentrations
of
trace
and
ultratrace
minerals
(
copper
,
manganese
,
molybdenum
,
selenium
,
zinc
)
and
plasma
retinol
and
alpha-tocopherol
were
in
the
reference
ranges
.
Vitamin
A
intakes
(
r
=
0
.
49
,
p
<
0
.
05
)
and
plasma
retinol-binding
protein
concentrations
(
r
=
0
.
42
,
p
<
0
.
05
)
were
positively
correlated
with
plasma
retinol
concentrations
at
3
mo
of
study
.
At
6
mo
,
concentrations
of
plasma
transthyretin
(
r
=
0
.
72
,
p
<
0
.
01
)
and
retinol-binding
protein
(
r
=
0
.
48
,
p
<
0
.
05
)
were
positively
correlated
with
plasma
retinol
concentrations
.
At
6
mo
,
concentrations
of
plasma
transthyretin
(
r
=
0
.
52
,
p
<
0
.
05
)
were
positively
correlated
with
retinol-binding
protein
concentrations
.
Phenex-
1
supports
normal
mean
iron
status
indices
and
mean
concentrations
of
trace
and
ultratrace
minerals
,
retinol
,
and
alpha-tocopherol
when
fed
in
adequate
amounts
.
Diseases
Validation
Diseases presenting
"complete blood count"
symptom
classical phenylketonuria
familial mediterranean fever
heparin-induced thrombocytopenia
holt-oram syndrome
oculocutaneous albinism
pyomyositis
severe combined immunodeficiency
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