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Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings.
[dentinogenesis imperfecta]
Osteogenesis
imperfecta
(
OI
)
is
a
hereditary
disorder
characterized
by
increased
tendency
for
bone
fractures
due
to
high
fragility
.
The
clinical
and
radiological
features
of
OI
manifest
in
different
age
groups
,
although
the
disease
is
congenital
in
nature
.
Besides
bone
fragility
,
features
like
laxity
of
the
ligaments
,
blue
sclera
,
growth
retardation
,
and
scoliosis
are
also
observed
.
In
severe
cases
,
respiratory
distress
and
death
have
been
reported
.
The
most
important
oral
finding
in
OI
is
the
presence
of
yellowish-
brown
-coloured
brittle
teeth
characteristic
of
dentinogenesis
imperfecta
.
Genetic
factors
play
a
very
important
role
in
the
pathogenesis
of
OI
either
as
a
dominant
or
recessive
factor
.
When
a
child
has
OI
,
there
is
a
25
%
chance
of
the
sibling
to
have
the
same
disorder
.
We
report
two
cases
of
OI
in
siblings
born
to
parents
with
a
history
of
consanguineous
marriage
.
The
clinical
and
radiological
features
of
the
two
cases
are
described
in
detail
.
