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Dento-maxillo-facial phenotype and implants-based oral rehabilitation in Ectodermal Dysplasia with WNT10A gene mutation: report of a case and literature review.
[oligodontia]
To
report
the
dento-
craniofacial
phenotype
of
a
family
affected
by
a
WNT
10
A
HED
and
to
describe
the
implant-based
oral
rehabilitation
of
a
patient
presenting
a
severe
oligodontia
linked
to
this
mutation
.
A
molecular
hypothesis
concerning
the
involvement
of
Wnt-β-catenin
pathway
in
implant
osteointegration
will
be
proposed
.
Patients
affected
by
a
WNT
10
A
mutation
were
included
from
a
large
group
of
HED
patients
.
WNT
10
A
gene
was
sequenced
in
second
intention
for
patients
negative
for
EDA
-
EDAR
-
EDARADD
mutations
.
Dento-
craniofacial
phenotype
was
described
based
on
clinical
and
radiological
data
.
Severe
oligodontia
was
observed
in
the
patient
affected
by
a
compound
heterozygous
mutation
of
WNT
10
A
gene
.
CT
exams
showed
marked
maxillary
bone
hypoplasia
in
the
posterior
areas
with
a
sub-normal
mandible
treatment
consisted
in
the
placement
of
4
mandibular
implants
and
in
2
implant-supported
bridges
.
In
the
maxilla
,
an
autogenous
bone
graft
was
indicated
.
The
post-operative
radiological
follow-up
showed
partial
bone
resorption
of
the
grafts
,
treated
with
ramus
bone
shaving
and
a
membrane
,
followed
by
the
placement
of
4
maxillary
implants
.
Patients
affected
by
WNT
10
A
HED
require
multi-disciplinary
dental
diagnosis
and
treatment
.
A
close
post-operative
radiological
follow-up
appears
necessary
given
the
biological
functions
of
Wnt-
β-catenin
in
bone
repair
.