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[Oral cavity features in patients suffering from osteogenesis imperfecta].
[dentinogenesis imperfecta]
Osteogenesis
Imperfecta
(
OI
)
is
a
rare
hereditary
connective
tissue
disorder
.
This
pathology
is
characterized
by
disruption
of
biosynthesis
of
Type
I
collagen
,
and
production
of
limited
amount
of
defective
and
imperfect
collagens
.
This
causes
decrease
in
bone
mass
of
human
body
,
bones
become
fragile
and
brittle
,
resulting
in
unreasonable
multiple
fractures
.
Reportedly
,
number
of
patients
with
OI
ranges
between
32
-
38
in
Georgia
.
However
,
exact
number
of
patients
,
including
children
and
their
parents
,
is
unknown
.
Dentinogenesis
Imperfecta
(
DI
;
DGI
)
and
skeletal
malocclusion
occupy
special
place
in
varied
spectrum
of
OI
clinical
symptoms
.
We
studied
14
patients
:
9
women
(
64
.
3
%
)
,
5
men
(
35
.
7
%
)
and
divided
them
in
three
age
groups
:
I
-
2
.
5
-
6
years
-
period
of
primary
dentition
(
28
.
6
%
)
,
II
-
6
-
14
years
-
period
of
changing
teeth
dentition
(
35
.
7
%
)
and
III
-
above
14
years
-
period
of
permanent
dentition
(
35
.
7
%
)
.
28
.
5
%
of
screened
patients
had
one
of
the
symptoms
of
DI
,
such
as
tooth
discoloration
.
Discoloration
of
primary
teeth
was
revealed
in
4
patients
(
primary
dentition
)
.
Another
symptom
of
DI
,
such
as
early
abrasion
,
was
detected
in
5
patients
i
.
e
.
35
.
71
%
.
This
was
divided
in
the
following
manner
:
I
age
group
-
3
cases
,
II
and
III
age
groups
-
1
-
1
cases
.
It
was
also
observed
that
early
abrasion
of
primary
teeth
prevails
over
permanent
.
One
of
DI
's
radiographic
symptoms
,
such
as
peculiar
form
of
teeth
crown
and
root
,
was
revealed
in
21
.
4
%
or
in
3
patients
,
2
of
whom
had
bulbous
crown
,
and
the
third
one
deformed
(
curved
)
root
.
Peculiar
characteristics
of
DI
,
such
as
increased
constriction
of
the
coronal
-radicular
junction
,
obliterated
pulp
chamber
,
short
and
narrow
roots
,
were
not
observed
in
the
patients
examined
.
Interesting
characteristic
of
DI
,
such
as
periapical
destruction
of
intact
tooth
root
,
was
revealed
in
the
form
of
bone
defect
in
7
.
1
%
of
those
examined
(
1
patient
)
.
Therefore
,
out
of
examined
14
patients
with
OI
-
DI
had
6
patients
or
42
.
85
%
of
cases
.
Also
,
interesting
observation
was
revealed
-
DI
is
more
common
in
primary
teeth
(
66
.
66
%
)
than
in
permanent
(
33
.
33
%
)
.
Radiographic
examination
-
orthopantomography
-
revealed
secondary
osteoporosis
of
jaw
bones
in
100
%
of
cases
.
Mucous
tissue
of
examined
patients
is
within
normal
range
.
Among
examined
patients
,
1
case
of
adenty
,
1
case
of
retention
and
1
case
of
overcomplex
tooth
were
revealed
.
According
to
current
literature
,
it
is
unknown
whether
there
is
a
lgical
relationship
between
adenty
,
retention
,
overcomplex
teeth
and
OI
.
This
will
be
defined
by
future
research
.
Diseases
Validation
Diseases presenting
"rare hereditary connective tissue disorder"
symptom
dentinogenesis imperfecta
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