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Neuropsychological outcome of experimental manipulation of phenylalanine intake in treated phenylketonuria.
[classical phenylketonuria]
Blood
phenylalanine
concentrations
were
experimentally
increased
for
3
months
by
means
of
a
phenylalanine-complemented
amino
acid
supplement
in
a
group
of
16
children
aged
10
-
16
years
with
classical
phenylketonuria
who
had
been
treated
early
and
who
had
remained
on
the
restricted
diet
.
Average
concentrations
achieved
during
challenge
were
between
1000
and
1300
mumol
/
L
.
Psychological
outcome
was
measured
by
a
neuropsychological
battery
consisting
of
tests
of
verbal
and
spatial
memory
,
attention
and
fine
motor
coordination
.
A
triple-blind
,
repeated
measures
,
randomized
,
crossover
design
was
adopted
to
control
for
practice
and
expectancy
effects
.
Subjects
were
assessed
at
baseline
and
at
the
end
of
the
first
and
second
phenylalanine
manipulation
periods
.
Significant
interactions
(
ANOVA
)
emerged
as
predicted
for
phenylalanine
concentrations
,
but
similar
crossover
effects
were
not
found
for
any
of
the
neuropsychological
tests
.
The
results
suggested
that
medium-term
hyperphenylalaninaemia
in
treated
PKU
is
not
harmful
to
psychological
functioning
in
older
children
and
adolescents
who
have
been
continuously
treated
up
to
and
beyond
age
10
years
,
though
the
susceptibility
of
executive
functions
needs
to
be
further
researched
.
The
findings
add
some
weight
to
the
idea
that
by
late
childhood
the
vulnerability
of
the
nervous
system
to
the
neurotoxic
influence
of
phenylalanine
may
be
much
reduced
.