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Classical phenylketonuria associated with Goldenhar's syndrome. A case report.
[classical phenylketonuria]
Classical
phenylketonuria
(
PKU
)
and
Goldenhar
's
syndrome
were
diagnosed
in
a
six
-
month
-old
male
infant
who
was
referred
to
Hacettepe
Children
's
Hospital
for
evaluation
of
developmental
delay
.
There
had
been
epibulbar
dermoids
in
his
left
eye
,
strabismus
,
bilateral
multiple
preauricular
appendices
,
malar
hypoplasia
,
micrognathia
,
hemifacial
microsoma
and
high
palatal
vault
.
In
addition
to
congenital
anomalies
and
developmental
delay
,
blond
hair
,
fair
skin
and
unusual
urinary
odor
were
noted
.
Ferric
chloride
test
on
his
urine
sample
was
positive
,
and
the
plasma
phenylalanine
level
was
high
(
34
mg
/
dl
)
.
Based
on
these
clinical
and
biochemical
findings
,
the
diagnoses
of
phenylketonuria
and
Goldenhar
's
syndrome
were
made
.
To
our
knowledge
,
this
is
the
first
case
with
PKU
and
Goldenhar
's
syndrome
.
Diseases
Validation
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"first case"
symptom
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