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Oral manifestations and dental management of a child with Zellweger syndrome.
[zellweger syndrome]
Zellweger
syndrome
(
ZS
)
is
a
rare
autosomal
recessive
disorder
,
resulting
from
an
impairment
in
peroxisome
function
.
It
is
characterized
by
craniofacial
dysmorphism
and
neurological
abnormalities
,
and
involves
several
systems
,
which
may
complicate
dental
and
anesthesia
management
.
The
case
of
a
7
-
year
-old
girl
diagnosed
with
ZS
is
described
with
emphasis
on
oral
manifestations
,
oral
rehabilitation
under
general
anesthesia
(
GA
)
,
and
home
oral
care
.
Apart
from
the
unique
features
of
ZS
,
she
presented
with
clinodactyly
,
distinctive
palatal
vault
,
Class
III
malocclusion
,
missing
teeth
,
microdontia
,
and
delayed
dental
formation
.
Dental
treatment
under
GA
was
conducted
with
concerns
of
risk
of
respiratory
insufficiency
.
Oral
home
care
by
the
parent
and
regular
recall
visits
were
essential
to
maintain
good
oral
health
.
Children
with
ZS
may
survive
into
late
childhood
.
They
,
however
,
present
multiple
health
problems
that
are
of
special
concern
for
not
only
the
pediatric
dentist
but
also
the
anesthesiologist
.
Collaboration
with
the
medical
team
is
essential
for
optimal
care
of
these
patients
.
Diseases
Validation
Diseases presenting
"oral care"
symptom
zellweger syndrome
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