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Oral features in five adult patients with Wolf-Hirschhorn syndrome.
[wolf-hirschhorn syndrome]
The
Wolf-
Hirschhorn
syndrome
(
WHS
)
is
a
rare
genetic
condition
presenting
with
severe
mental
disability
,
growth
retardation
,
muscular
hypotonia
,
seizures
,
craniofacial
abnormalities
and
defects
in
the
cardiovascular
,
genitourinary
and
digestive
apparata
.
To
date
,
few
data
about
oral
status
of
afflicted
patients
were
reported
,
and
this
syndrome
is
still
unfamiliar
among
dental
and
maxillofacial
professionals
.
Aim
of
the
present
case
series
was
to
provide
oral
data
from
five
patients
with
WHS
(
3
women
and
2
men
)
aged
19
-
41
years
.
All
patients
entered
in
a
long
-stay
Institution
with
an
inner
dental
service
in
1998
and
underwent
regular
dental
examination
and
hygiene
treatment
.
Neither
tooth
agenesis
or
dental
size
and
shape
anomalies
were
found
in
the
analyzed
subjects
except
for
one
man
showing
multiple
cone-shaped
teeth
.
At
the
beginning
,
bad
dental
and
periodontal
conditions
with
gingival
signs
and
recurrent
mucosal
inflammation
were
found
in
all
patients
.
After
motivation
of
their
tutors
,
dental
and
periodontal
parameters
were
recorded
during
periodic
assessments
and
a
large
decrease
in
the
gingival
index
was
found
over
time
.
These
data
could
aware
dentists
about
the
therapeutic
modalities
to
improve
oral
health
of
WHS
patients
.
Diseases
Validation
Diseases presenting
"severe mental disability"
symptom
wolf-hirschhorn syndrome
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