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Iron status and iron supplementation in children with classical phenylketonuria.
[classical phenylketonuria]
Iron
(
Fe
)
status
and
Fe
supplementation
were
assessed
in
20
children
with
phenylketonuria
(
PKU
)
through
dietary
intake
and
through
measurements
of
ferritin
,
hematocrit
,
hemoglobin
,
mean
cellular
hemoglobin
,
mean
cellular
volume
,
serum
Fe
,
total
iron
binding
capacity
,
unbounded
iron
binding
capacity
,
transferrin
saturation
and
transferrin
.
Findings
were
compared
to
reference
values
and
to
data
from
age-matched
controls
.
The
prescribed
phenylalanine-
restricted
diet
supplied
all
the
recommended
nutrients
.
Dietary
Fe
was
present
in
the
diets
,
but
its
bioavailability
is
questionable
as
several
laboratory
results
were
not
within
accepted
reference
values
.
A
ferrous
sulphate
supplement
(
5
mg
elemental
Fe
/
kg
daily
)
was
given
for
120
days
to
a
group
of
PKU
children
with
lower
Fe
parameters
,
thus
changing
some
of
the
parameters
studied
.
Serum
ferritin
(
p
<
0
.
1
)
,
transferrin
saturation
and
serum
Fe
(
p
<
0
.
05
)
increased
after
the
treatment
.
The
need
for
improved
diagnosis
of
Fe
status
and
determination
of
whether
PKU
children
can
benefit
from
therapeutic
Fe
is
discussed
.
Diseases
Validation
Diseases presenting
"prescribed phenylalanine-restricted diet supplied all the recommended nutrients"
symptom
classical phenylketonuria
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