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Growth and body composition in children with classical phenylketonuria: results in 34 patients and review of the literature.
[classical phenylketonuria]
Treatment
of
phenylketonuria
(
PKU
,
OMIM
261600
)
means
a
diet
restricted
in
natural
protein
and
supplemented
with
phenylalanine
(
Phe
)
-
free
L-
amino
acid
mixtures
.
Growth
impairment
has
been
described
even
in
patients
with
a
total
protein
intake
at
or
above
the
recommended
dietary
allowance
(
RDA
)
.
In
the
present
study
,
growth
and
body
composition
(
fat-free
mass
(
FFM
)
and
fat
)
were
recorded
over
12
months
in
34
treated
PKU
patients
(
mean
age
8
.
7
years
at
baseline
)
.
Measurements
were
compared
with
those
of
healthy
peers
and
with
general
population
standard
(
Z-
)
scores
calculated
using
the
LMS
method
.
In
28
PKU
patients
,
data
on
birth
weight
and
birth
length
were
available
and
related
to
measurements
at
baseline
of
the
study
.
Mean
total
protein
intake
in
PKU
patients
was
124
%
(
range
77
-
193
%
)
of
the
RDA
(
DACH
2000
)
.
No
significant
differences
in
growth
and
body
composition
were
present
between
PKU
patients
and
healthy
populations
either
at
birth
or
during
the
study
period
.
The
significant
correlation
of
FFM
(
representing
muscle
mass
)
with
intake
of
natural
protein--rather
than
total
protein--indicates
that
the
enhancement
of
tolerance
to
natural
protein
may
be
of
value
in
PKU
patients
.
