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Oral manifestations of patients with Kenny-Caffey Syndrome.
[oligodontia]
Kenny-
Caffey
syndrome
(
KCS
)
is
a
rare
osteosclerotic
bone
dysplasia
characterized
by
hypocalcemia
,
short
stature
,
ophthalmological
features
,
and
teeth
anomalies
.
The
TBCE
gene
coding
for
a
tubulin-
specific
chaperone
E
,
is
located
at
chromosome
1
q
42
-
q
43
,
and
is
responsible
for
the
recessive
form
.
After
reviewing
the
literature
,
we
found
around
60
cases
,
however
with
limited
dental
data
.
In
this
article
5
new
individuals
with
KCS
,
are
described
focusing
on
oral
findings
.
All
cases
had
short
roots
and
showed
dental
anomalies
as
hypo
/
oligodontia
,
microdontia
.
Dental
anomalies
are
a
constant
feature
in
KCS
,
further
study
is
required
to
better
delineate
the
syndrome
.
Diseases
Validation
Diseases presenting
"rare osteosclerotic bone dysplasia"
symptom
oligodontia
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