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Clinical aspects, imaging features, and considerations on bisphosphonate-related osteonecrosis risk in a pediatric patient with osteogenesis imperfecta.
[dentinogenesis imperfecta]
Osteogenesis
imperfecta
(
OI
)
is
a
rare
hereditary
condition
caused
by
changes
in
collagen
metabolism
.
It
is
classified
into
four
types
according
to
clinical
,
genetic
,
and
radiological
criteria
.
Clinically
,
bone
fragility
,
short
stature
,
blue
sclerae
,
and
locomotion
difficulties
may
be
observed
in
this
disease
.
OI
is
often
associated
to
severe
dental
problems
,
such
as
dentinogenesis
imperfecta
(
DI
)
and
malocclusions
.
Radiographically
,
affected
teeth
may
have
crowns
with
bulbous
appearance
,
accentuated
constriction
in
the
cementoenamel
junction
,
narrowed
roots
,
large
root
canals
due
to
defective
dentin
formation
,
and
taurodontism
(
enlarged
pulp
chambers
)
.
There
is
no
definitive
cure
,
but
bisphosphonate
therapy
is
reported
to
improve
bone
quality
;
however
,
there
is
a
potential
risk
of
bisphosphonate-related
osteonecrosis
of
the
jaw
.
In
this
study
we
report
a
case
of
OI
in
a
male
pediatric
patient
with
no
family
history
of
OI
who
was
receiving
ongoing
treatment
with
intravenous
perfusion
of
bisphosphonate
and
who
required
dental
surgery
.
In
addition
,
we
discussed
the
clinical
and
imaging
findings
and
briefly
reviewed
the
literature
.
Diseases
Validation
Diseases presenting
"defective dentin formation"
symptom
dentin dysplasia
dentinogenesis imperfecta
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