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Dentinogenesis imperfecta in children with osteogenesis imperfecta: a clinical and ultrastructural study.
[dentinogenesis imperfecta]
The
aim
of
this
study
was
to
assess
the
correlation
between
osteogenesis
imperfecta
(
OI
)
and
dentinogenesis
imperfecta
(
DI
)
from
both
a
clinical
and
histological
point
of
view
,
particularly
clarifying
the
structural
and
ultrastructural
dentine
changes
.
Sixteen
children
(
6
-
12
years
aged
)
with
diagnosis
of
OI
were
examined
for
dental
alterations
referable
to
DI
.
For
each
patient
,
the
OI
type
(
I
,
III
,
or
IV
)
was
recorded
.
Extracted
or
normally
exfoliated
primary
teeth
were
subjected
to
a
histological
examination
(
to
both
optical
microscopy
and
confocal
laser-scanning
microscopy
)
.
A
total
of
ten
patients
had
abnormal
discolourations
referable
to
DI
:
four
patients
were
affected
by
OI
type
I
,
three
patients
by
OI
type
III
,
and
three
patients
by
OI
type
IV
.
The
discolourations
,
yellow
/
brown
or
opalescent
grey
,
could
not
be
related
to
the
different
types
of
OI
.
Histological
exam
of
primary
teeth
showed
severe
pathological
change
in
the
dentin
,
structured
into
four
different
layers
.
A
collagen
defect
due
to
odontoblast
dysfunction
was
theorized
to
be
on
the
base
of
the
histological
changes
.
There
is
no
correlation
between
the
type
of
OI
and
the
type
of
discolouration
.
The
underlying
dentinal
defect
seems
to
be
related
to
an
odontoblast
dysfunction
.
Diseases
Validation
Diseases presenting
"dental alterations"
symptom
dentinogenesis imperfecta
kabuki syndrome
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