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Variability in phenylalanine control predicts IQ and executive abilities in children with phenylketonuria.
[phenylketonuria]
A
number
of
studies
have
revealed
significant
relationships
between
cognitive
performance
and
average
phenylalanine
(
Phe
)
levels
in
children
with
phenylketonuria
(
PKU
)
,
but
only
a
few
studies
have
been
conducted
to
examine
the
relationships
between
cognitive
performance
and
variability
(
fluctuations
)
in
Phe
levels
.
In
the
current
study
,
we
examined
a
variety
of
indices
of
Phe
control
to
determine
which
index
best
predicted
IQ
and
executive
abilities
in
47
school
-age
children
with
early
-
and
continuously-treated
PKU
.
Indices
of
Phe
control
were
mean
Phe
,
the
index
of
dietary
control
,
change
in
Phe
with
age
,
and
several
indices
of
variability
in
Phe
(
standard
deviation
,
standard
error
of
estimate
,
and
percentage
of
spikes
)
.
These
indices
were
computed
over
the
lifetime
and
during
3
developmental
epochs
(
<
5
,
5
.
0
-
9
.
9
,
and
≥
10
years
of
age
)
.
Results
indicated
that
variability
in
Phe
was
generally
a
stronger
predictor
of
cognitive
performance
than
other
indices
of
Phe
control
.
In
addition
,
executive
performance
was
better
predicted
by
variability
in
Phe
during
older
than
younger
developmental
epochs
.
These
results
indicate
that
variability
in
Phe
should
be
carefully
controlled
to
maximize
cognitive
outcomes
and
that
Phe
control
should
not
be
liberalized
as
children
with
PKU
age
.
Diseases
Validation
Diseases presenting
"that variability in phe was generally a stronger predictor of cognitive performance than other indices of phe control"
symptom
phenylketonuria
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