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Long-term follow-up of children with classical phenylketonuria after diet discontinuation: a review.
[classical phenylketonuria]
The
age
at
which
children
suffering
from
classical
phenylketonuria
can
safely
discontinue
their
dietary
therapy
has
been
constantly
disputed
over
the
past
decades
.
Recently
,
most
phenylketonuria
centers
have
begun
to
recommend
a
life-
long
diet
,
especially
for
female
patients
.
Male
patients
are
also
advised
to
continue
their
diet
until
at
least
well
into
adult
age
.
As
a
result
of
this
new
outlook
in
therapy
management
,
we
reviewed
the
existing
literature
and
summarized
all
relevant
long
-term
follow-up
data
of
children
who
discontinued
their
debts
at
an
early
age
,
focusing
on
intellectual
and
neurological
performance
.
The
abilities
of
these
children
are
compared
during
dietary
treatment
and
again
several
years
after
diet
discontinuation
.
Results
show
clearly
that
children
maintaining
their
diets
into
their
teens
have
fewer
deficits
than
do
those
terminating
their
diets
before
10
years
of
age
.
It
seems
essential
to
initiate
diet
early
,
and
to
keep
blood
phenylalanine
levels
<
600
mumol
/
L
and
well
controlled
to
at
least
age
10
to
ensure
satisfactory
long
-term
development
of
the
patient
.
Furthermore
,
it
seems
highly
justified
to
maintain
a
life-
long
diet
which
can
be
liberalized
,
but
not
completely
discontinued
in
adulthood
.
Diseases
Validation
Diseases presenting
"relevant long-term follow-up data"
symptom
classical phenylketonuria
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