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Dentinal dysplasia type I: report of a case.
[dentin dysplasia]
A
case
of
dentinal
dysplasia
type
I
is
presented
.
This
rare
hereditary
disturbance
of
dentine
is
characterized
by
short
-rooted
teeth
with
sharp
conical
apical
constrictions
,
aberrant
growth
of
dentine
in
the
pulp
chamber
leading
to
reduced
pulp
space
in
permanent
teeth
and
total
pulpal
obliteration
in
the
primary
dentition
.
Clinical
,
radiographic
and
histopathological
material
from
a
7
-
year
-old
boy
,
showing
the
typical
features
of
this
disorder
in
which
teeth
are
prematurely
lost
through
periapical
abscesses
,
cysts
or
spontaneous
exfoliation
,
is
described
.
A
review
of
the
theories
of
pathogenesis
of
this
condition
is
included
.
Management
of
patients
with
dentinal
dysplasia
is
difficult
and
a
discussion
of
the
shortcomings
of
various
treatment
strategies
,
including
conventional
endodontic
therapy
,
periapical
curettage
and
retrograde
root
filling
,
and
a
preventive
regimen
,
are
discussed
.
In
this
case
,
despite
diagnosis
being
made
at
an
early
age
and
the
provision
of
regular
dental
care
,
the
patient
is
now
losing
teeth
because
of
spontaneous
abscess
formation
.
Diseases
Validation
Diseases presenting
"early age"
symptom
achondroplasia
aniridia
classical phenylketonuria
cohen syndrome
congenital adrenal hyperplasia
cowden syndrome
dentin dysplasia
junctional epidermolysis bullosa
kabuki syndrome
megacystis-microcolon-intestinal hypoperistalsis syndrome
papillon-lefèvre syndrome
primary hyperoxaluria type 1
severe combined immunodeficiency
werner syndrome
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