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Ultrastructure of dentin matrix in heritable dentin defects.
[dentin dysplasia]
Heritable
dentin
defects
form
a
group
of
diseases
which
exclusively
affect
dentin
among
the
various
dental
tissues
.
While
one
type
is
associated
with
the
generalized
connective
tissue
disorder
,
osteogenesis
imperfecta
,
other
types
occur
as
single
traits
.
The
clinical
manifestations
of
the
dentin
defects
vary
from
insignificant
to
severe
enough
to
cause
aesthetical
and
functional
failure
of
the
teeth
.
Scanning
and
transmission
electron
microscopic
studies
,
reviewed
in
this
paper
,
have
markedly
clarified
the
ultrastructure
of
the
aberrant
dentin
matrix
.
Both
similar
and
different
changes
seem
to
occur
in
the
various
forms
of
heritable
dentin
defects
.
Abnormalities
in
the
appearance
and
organization
pattern
of
collagen
fibers
in
the
defective
dentin
partly
resemble
those
observed
in
skin
in
generalized
connective
tissue
diseases
.
The
similarity
of
ultrastructural
findings
in
dentin
defects
,
which
are
currently
classified
as
distinct
entities
,
and
even
in
diseases
affecting
other
tissues
,
could
be
related
to
the
complicated
interactions
between
the
extracellular
matrix
macromolecules
.
Thus
,
many
of
the
changes
observed
may
be
secondary
in
nature
.
Ultrastructural
studies
can
help
us
to
understand
the
pathogenesis
of
the
different
types
of
heritable
dentin
defects
as
well
as
aid
in
diagnostics
and
classification
of
these
diseases
.
Diseases
Validation
Diseases presenting
"the generalized connective tissue disorder"
symptom
dentin dysplasia
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