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Craniofacial and dental features in kabuki syndrome patients.
[kabuki syndrome]
Objective
:
To
describe
oral
manifestations
in
Brazilian
individuals
with
Kabuki
syndrome
,
a
multiple
congenital
anomaly
/
mental
retardation
syndrome
.
Study
Design
:
A
total
of
16
Kabuki
syndrome
individuals
,
aged
between
8
to
24
Â
years
and
of
both
sexes
,
were
referred
by
the
Department
of
Clinical
Genetics
for
oral
treatment
and
follow-up
to
the
Oral
Care
Center
for
Inherited
Diseases
,
University
Hospital
of
BrasÃlia
,
BrasÃlia
,
Brazil
.
Each
individual
underwent
complete
physical
examination
,
as
well
as
intraoral
and
radiographic
examinations
.
Results
:
Craniofacial
and
dental
alterations
were
observed
in
all
Kabuki
syndrome
patients
examined
.
In
addition
,
atypical
shape
of
the
molars
'
crowns
,
occlusal
convergence
of
the
premolars
'
crowns
,
and
root
dilaceration
were
also
observed
.
Enamel
diffuse
opacities
were
observed
in
permanent
dentition
(
n
 
=
 
10
)
.
Conclusion
:
A
great
clinical
heterogeneity
was
observed
in
Kabuki
syndrome
individuals
in
line
with
previous
studies
in
the
literature
.
Further
clinical
and
molecular
studies
are
necessary
in
order
to
better
understand
the
presence
of
dental
anomalies
in
this
syndrome
.
Diseases
Validation
Diseases presenting
"dental anomalies in this syndrome"
symptom
kabuki syndrome
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