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Brittle teeth with brittle bone in a family for four generations: Case report and literature review.
[dentinogenesis imperfecta]
Dentinogenesis
imperfect
(
DI
)
is
a
hereditary
dentine
disorder
affecting
both
deciduous
and
permanent
teeth
.
DI
is
caused
by
mutations
in
genes
encoding
for
type
I
collagen
leading
to
discoloration
of
teeth
.
Shield
around
30
years
ago
classified
DI
into
3
types
(
type
I
,
II
,
and
III
)
.
DI
type
I
is
associated
with
osteogenesis
imperfect
(
OI
)
,
which
is
an
inheritable
disorder
of
connective
tissue
.
Bone
fragility
and
fracture
of
bone
with
minor
trauma
are
hallmarks
of
this
disorder
.
The
objective
of
this
article
is
to
report
and
review
a
rare
case
of
DI
with
OI
affecting
4
generations
of
the
family
.
Through
this
article
,
we
intend
to
highlight
genetic
influence
that
affected
a
family
for
many
generations
,
discuss
the
oral
manifestations
that
can
lead
to
the
diagnosis
of
OI
,
and
the
importance
of
early
diagnosis
of
OI
.
Diseases
Validation
Diseases presenting
"fracture of bone with minor trauma are hallmarks of this disorder"
symptom
dentinogenesis imperfecta
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